DOI: 10.1148/rg.276075138
Pediatric Orbit Tumors and Tumorlike Lesions: Nonosseous Lesions of the Extraocular Orbit1
Ellen M. Chung, LTC, MC, USA,
James G. Smirniotopoulos, MD,
Charles S. Specht, MD,
Jason W. Schroeder, LCDR, MC, USN, and
Regino Cube, 2LT, MC, USA
1 From the Department of Radiologic Pathology (E.M.C.) and Ophthalmic Pathology Section, Department of Neuropathology (C.S.S.), Armed Forces Institute of Pathology, 6825 16th St NW, Washington, DC 20306-6000; Department of Radiology and Radiological Sciences, Edward F. Hebert School of Medicine, Uniformed University of the Health Sciences, Bethesda, Md (J.G.S., R.C.); National Capitol Radiology Consortium, National Naval Medical Center, Bethesda, Md (J.W.S.); and Department of Radiology, Walter Reed Army Medical Center, Washington, DC (J.W.S.). Received June 18, 2007; revision requested July 19 and received July 31; accepted August 3. All authors have no financial relationships to disclose.
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Figure 1a. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 1b. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 1c. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 1d. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 1e. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 1f. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 1g. Embryonal rhabdomyosarcoma with alveolar features in an 18-year-old boy with left proptosis. (a) Sagittal T1-weighted magnetic resonance (MR) image shows a well-circumscribed extraconal mass (arrowhead) superior to the ocular globe and isointense relative to muscle. (b) Another sagittal MR image shows that the mass is separate from the superior rectus muscle (arrow). (c) On the axial T2-weighted image, the mass is heterogeneous in signal intensity and predominantly hyperintense relative to gray matter and muscle. (d) Coronal T1-weighted image obtained after intravenous administration of gadolinium-based contrast material reveals intense enhancement of the tumor. (e) Photograph of the gross specimen shows it to be a well-circumscribed, fleshy tumor. Scale is in centimeters. (f) Photomicrograph (original magnification x200; hematoxylin-eosin [H-E] stain) shows pleomorphic (spindle-shaped and round) rhabdomyosarcomatous cells arranged in an alveolar pattern within a collagenized stroma. (g) Higher power photomicrograph (original magnification x400; H-E stain) of the same tumor specimen but from another area shows neoplastic cells with hyperchromatic, pleomorphic nuclei and scant cytoplasm distributed through a myxoid matrix. Fibrillary structures suggestive of cross striations may be seen in rare cells that have more ample cytoplasm and vesicular nuclei (straight arrow). Note numerous mitotic figures (arrowheads) and occasional pyknotic nuclei (tailed arrow).
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Figure 2a. Rhabdomyosarcoma involving the orbit and maxillary sinus in an 18-year-old girl with erythema of the right lower eyelid followed by proptosis 2 months later. (a) Unenhanced coronal computed tomographic (CT) image (soft-tissue window) demonstrates a large, irregular soft-tissue mass in the maxillary sinus and inferior orbit that is iso- to slightly hyperattenuating relative to muscle. There is marked destruction of the bony orbital floor (arrowhead). (b) Same image, shown with a bone window level, better depicts the bone destruction (arrowhead).
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Figure 2b. Rhabdomyosarcoma involving the orbit and maxillary sinus in an 18-year-old girl with erythema of the right lower eyelid followed by proptosis 2 months later. (a) Unenhanced coronal computed tomographic (CT) image (soft-tissue window) demonstrates a large, irregular soft-tissue mass in the maxillary sinus and inferior orbit that is iso- to slightly hyperattenuating relative to muscle. There is marked destruction of the bony orbital floor (arrowhead). (b) Same image, shown with a bone window level, better depicts the bone destruction (arrowhead).
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Figure 3a. Rhabdomyosarcoma in a 4-year-old boy with sickle cell anemia. (a) Coronal T1-weighted image shows a mass that is isointense relative to white matter involving the medial left orbit and the adjacent epidural space of the anterior cranial fossa (arrowhead). (b) Coronal T2-weighted MR image demonstrates hyperintense, left retropharyngeal (arrow) and left cervical (arrowhead) lymph nodes. Biopsy specimens from the latter revealed rhabdomyosarcoma. (c) Axial CT image shows soft tissue in the left maxillary sinus and periosteal reaction of the anterior wall of the sinus (arrow).
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Figure 3b. Rhabdomyosarcoma in a 4-year-old boy with sickle cell anemia. (a) Coronal T1-weighted image shows a mass that is isointense relative to white matter involving the medial left orbit and the adjacent epidural space of the anterior cranial fossa (arrowhead). (b) Coronal T2-weighted MR image demonstrates hyperintense, left retropharyngeal (arrow) and left cervical (arrowhead) lymph nodes. Biopsy specimens from the latter revealed rhabdomyosarcoma. (c) Axial CT image shows soft tissue in the left maxillary sinus and periosteal reaction of the anterior wall of the sinus (arrow).
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Figure 3c. Rhabdomyosarcoma in a 4-year-old boy with sickle cell anemia. (a) Coronal T1-weighted image shows a mass that is isointense relative to white matter involving the medial left orbit and the adjacent epidural space of the anterior cranial fossa (arrowhead). (b) Coronal T2-weighted MR image demonstrates hyperintense, left retropharyngeal (arrow) and left cervical (arrowhead) lymph nodes. Biopsy specimens from the latter revealed rhabdomyosarcoma. (c) Axial CT image shows soft tissue in the left maxillary sinus and periosteal reaction of the anterior wall of the sinus (arrow).
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Figure 4a. Embryonal rhabdomyosarcoma in a 4-year-old girl with right proptosis. (a) Axial T1-weighted MR image demonstrates a well-defined mass that is iso- to slightly hypointense relative to muscle (arrowhead). Note the swelling of the upper eyelid. (b) Axial T1-weighted image with fat saturation shows intense, rimlike enhancement (arrowhead) of the mass and overlying lid.
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Figure 4b. Embryonal rhabdomyosarcoma in a 4-year-old girl with right proptosis. (a) Axial T1-weighted MR image demonstrates a well-defined mass that is iso- to slightly hypointense relative to muscle (arrowhead). Note the swelling of the upper eyelid. (b) Axial T1-weighted image with fat saturation shows intense, rimlike enhancement (arrowhead) of the mass and overlying lid.
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Figure 5a. Rhabdomyosarcoma with imaging features mimicking those of hemangioma in a 4-year-old boy with right eye pain and proptosis. (a) Axial CT image shows a mass isoattenuating relative to muscle with a lobular contour suggestive of hemangioma. (b) Coronal short inversion time inversion-recovery image reveals the hyperintense mass, which contains serpentine dark flow void (arrow). Note the ill-defined inferior tumor margin (arrowhead). (c) Sagittal T2-weighted image shows another flow void (arrow), mimicking the features of hemangioma. (d) Coronal T1-weighted image with fat saturation shows somewhat heterogeneous enhancement, which is more characteristic of rhabdomyosarcoma than hemangioma. Note the encased optic nerve (arrowhead). Because the imaging features are nondiagnostic and because the patient age is inconsistent with hemangioma, biopsy was performed to obtain the proper diagnosis.
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Figure 5b. Rhabdomyosarcoma with imaging features mimicking those of hemangioma in a 4-year-old boy with right eye pain and proptosis. (a) Axial CT image shows a mass isoattenuating relative to muscle with a lobular contour suggestive of hemangioma. (b) Coronal short inversion time inversion-recovery image reveals the hyperintense mass, which contains serpentine dark flow void (arrow). Note the ill-defined inferior tumor margin (arrowhead). (c) Sagittal T2-weighted image shows another flow void (arrow), mimicking the features of hemangioma. (d) Coronal T1-weighted image with fat saturation shows somewhat heterogeneous enhancement, which is more characteristic of rhabdomyosarcoma than hemangioma. Note the encased optic nerve (arrowhead). Because the imaging features are nondiagnostic and because the patient age is inconsistent with hemangioma, biopsy was performed to obtain the proper diagnosis.
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Figure 5c. Rhabdomyosarcoma with imaging features mimicking those of hemangioma in a 4-year-old boy with right eye pain and proptosis. (a) Axial CT image shows a mass isoattenuating relative to muscle with a lobular contour suggestive of hemangioma. (b) Coronal short inversion time inversion-recovery image reveals the hyperintense mass, which contains serpentine dark flow void (arrow). Note the ill-defined inferior tumor margin (arrowhead). (c) Sagittal T2-weighted image shows another flow void (arrow), mimicking the features of hemangioma. (d) Coronal T1-weighted image with fat saturation shows somewhat heterogeneous enhancement, which is more characteristic of rhabdomyosarcoma than hemangioma. Note the encased optic nerve (arrowhead). Because the imaging features are nondiagnostic and because the patient age is inconsistent with hemangioma, biopsy was performed to obtain the proper diagnosis.
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Figure 5d. Rhabdomyosarcoma with imaging features mimicking those of hemangioma in a 4-year-old boy with right eye pain and proptosis. (a) Axial CT image shows a mass isoattenuating relative to muscle with a lobular contour suggestive of hemangioma. (b) Coronal short inversion time inversion-recovery image reveals the hyperintense mass, which contains serpentine dark flow void (arrow). Note the ill-defined inferior tumor margin (arrowhead). (c) Sagittal T2-weighted image shows another flow void (arrow), mimicking the features of hemangioma. (d) Coronal T1-weighted image with fat saturation shows somewhat heterogeneous enhancement, which is more characteristic of rhabdomyosarcoma than hemangioma. Note the encased optic nerve (arrowhead). Because the imaging features are nondiagnostic and because the patient age is inconsistent with hemangioma, biopsy was performed to obtain the proper diagnosis.
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Figure 6a. Orbital cellulitis in a 5-year-old boy with periorbital erythema. (a) Axial CT image shows increased attenuation in the preseptal and postseptal fat, a finding consistent with inflammation. Note the soft-tissue opacification of the ethmoid air cells and sphenoid sinus. (b) Coronal contrast-enhanced CT image shows a subperiosteal abscess (arrowhead) along the lamina papyracea. The rim enhancement seen here is rare in rhabdomyosarcoma.
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Figure 6b. Orbital cellulitis in a 5-year-old boy with periorbital erythema. (a) Axial CT image shows increased attenuation in the preseptal and postseptal fat, a finding consistent with inflammation. Note the soft-tissue opacification of the ethmoid air cells and sphenoid sinus. (b) Coronal contrast-enhanced CT image shows a subperiosteal abscess (arrowhead) along the lamina papyracea. The rim enhancement seen here is rare in rhabdomyosarcoma.
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Figure 7a. Infantile hemangioma in a 2-month-old infant who was noted to have a mass in the left temporal area shortly after birth. (a) Coronal T2-weighted image shows a mass slightly hyperintense relative to muscle and brain that contains numerous black flow voids (arrowheads). (b) Axial T2-weighted image shows that the mass extends into the temporal region. Note the prominent intratumoral flow voids (arrowhead). (c) Photograph of the gross specimen reveals a red, hemorrhagic tumor with circumscribed borders. Scale is in centimeters. (d) Photograph of the gross specimen from another patient with a capillary hemangioma of the scalp shows a red, vascular mass with lobulated contour.
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Figure 7b. Infantile hemangioma in a 2-month-old infant who was noted to have a mass in the left temporal area shortly after birth. (a) Coronal T2-weighted image shows a mass slightly hyperintense relative to muscle and brain that contains numerous black flow voids (arrowheads). (b) Axial T2-weighted image shows that the mass extends into the temporal region. Note the prominent intratumoral flow voids (arrowhead). (c) Photograph of the gross specimen reveals a red, hemorrhagic tumor with circumscribed borders. Scale is in centimeters. (d) Photograph of the gross specimen from another patient with a capillary hemangioma of the scalp shows a red, vascular mass with lobulated contour.
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Figure 7c. Infantile hemangioma in a 2-month-old infant who was noted to have a mass in the left temporal area shortly after birth. (a) Coronal T2-weighted image shows a mass slightly hyperintense relative to muscle and brain that contains numerous black flow voids (arrowheads). (b) Axial T2-weighted image shows that the mass extends into the temporal region. Note the prominent intratumoral flow voids (arrowhead). (c) Photograph of the gross specimen reveals a red, hemorrhagic tumor with circumscribed borders. Scale is in centimeters. (d) Photograph of the gross specimen from another patient with a capillary hemangioma of the scalp shows a red, vascular mass with lobulated contour.
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Figure 7d. Infantile hemangioma in a 2-month-old infant who was noted to have a mass in the left temporal area shortly after birth. (a) Coronal T2-weighted image shows a mass slightly hyperintense relative to muscle and brain that contains numerous black flow voids (arrowheads). (b) Axial T2-weighted image shows that the mass extends into the temporal region. Note the prominent intratumoral flow voids (arrowhead). (c) Photograph of the gross specimen reveals a red, hemorrhagic tumor with circumscribed borders. Scale is in centimeters. (d) Photograph of the gross specimen from another patient with a capillary hemangioma of the scalp shows a red, vascular mass with lobulated contour.
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Figure 8a. Capillary hemangioma in an 8-week-old girl with a 2-week history of left proptosis. (a, b) Axial contrast-enhanced CT images (a obtained at a lower level than b) show an intensely enhancing intraconal mass in the left orbit. (c) Photomicrograph (original magnification x100; H-E stain) demonstrates well-circumscribed lobules of neoplastic cells (*). (d) Photomicrograph (original magnification x400; H-E stain) shows small vascular spaces (arrowheads) surrounded by spindle-shaped stromal cells.
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Figure 8b. Capillary hemangioma in an 8-week-old girl with a 2-week history of left proptosis. (a, b) Axial contrast-enhanced CT images (a obtained at a lower level than b) show an intensely enhancing intraconal mass in the left orbit. (c) Photomicrograph (original magnification x100; H-E stain) demonstrates well-circumscribed lobules of neoplastic cells (*). (d) Photomicrograph (original magnification x400; H-E stain) shows small vascular spaces (arrowheads) surrounded by spindle-shaped stromal cells.
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Figure 8c. Capillary hemangioma in an 8-week-old girl with a 2-week history of left proptosis. (a, b) Axial contrast-enhanced CT images (a obtained at a lower level than b) show an intensely enhancing intraconal mass in the left orbit. (c) Photomicrograph (original magnification x100; H-E stain) demonstrates well-circumscribed lobules of neoplastic cells (*). (d) Photomicrograph (original magnification x400; H-E stain) shows small vascular spaces (arrowheads) surrounded by spindle-shaped stromal cells.
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Figure 8d. Capillary hemangioma in an 8-week-old girl with a 2-week history of left proptosis. (a, b) Axial contrast-enhanced CT images (a obtained at a lower level than b) show an intensely enhancing intraconal mass in the left orbit. (c) Photomicrograph (original magnification x100; H-E stain) demonstrates well-circumscribed lobules of neoplastic cells (*). (d) Photomicrograph (original magnification x400; H-E stain) shows small vascular spaces (arrowheads) surrounded by spindle-shaped stromal cells.
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Figure 9. Involuting hemangioma in a 5-week-old girl born with a large right orbital mass. Photomicrograph (original magnification x100; H-E stain) reveals that the stroma is more collagenized (*) between the islands of neoplastic cells than is seen in less mature lobular capillary hemangiomas (cf Fig 8).
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Figure 10a. Capillary hemangioma in a 9-week-old girl with right exophthalmos. (a) Axial T1-weighted image shows the lobular contour of an intraconal mass (arrowhead) with signal intensity similar to that of muscle and contrasted against the hyperintense conal fat. (b) Axial contrast-enhanced T1-weighted image with fat saturation demonstrates diffuse intense enhancement of the lobular mass. (c) Sagittal T2-weighted image also shows the hyperintense mass, which contains flow voids (arrowhead).
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Figure 10b. Capillary hemangioma in a 9-week-old girl with right exophthalmos. (a) Axial T1-weighted image shows the lobular contour of an intraconal mass (arrowhead) with signal intensity similar to that of muscle and contrasted against the hyperintense conal fat. (b) Axial contrast-enhanced T1-weighted image with fat saturation demonstrates diffuse intense enhancement of the lobular mass. (c) Sagittal T2-weighted image also shows the hyperintense mass, which contains flow voids (arrowhead).
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Figure 10c. Capillary hemangioma in a 9-week-old girl with right exophthalmos. (a) Axial T1-weighted image shows the lobular contour of an intraconal mass (arrowhead) with signal intensity similar to that of muscle and contrasted against the hyperintense conal fat. (b) Axial contrast-enhanced T1-weighted image with fat saturation demonstrates diffuse intense enhancement of the lobular mass. (c) Sagittal T2-weighted image also shows the hyperintense mass, which contains flow voids (arrowhead).
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Figure 11. Venous-lymphatic malformation in a 9-year-old-girl with left proptosis and a left medial canthal mass. Photograph shows the clinical appearance of the eye, with soft red lobules extending over the eye.
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Figure 12a. Venous-lymphatic malformation in a 9-year-old girl. (a) Axial unenhanced CT image reveals a medial soft-tissue attenuation mass with calcification (arrowhead). (b) Photomicrograph (original magnification x100; H-E stain) shows a thin-walled vascular space that contains serous fluid (*); note the lymphoid aggregates (arrows) and scattered foci of brown hemosiderin (arrowheads).
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Figure 12b. Venous-lymphatic malformation in a 9-year-old girl. (a) Axial unenhanced CT image reveals a medial soft-tissue attenuation mass with calcification (arrowhead). (b) Photomicrograph (original magnification x100; H-E stain) shows a thin-walled vascular space that contains serous fluid (*); note the lymphoid aggregates (arrows) and scattered foci of brown hemosiderin (arrowheads).
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Figure 13a. Venous-lymphatic malformation of the orbit and face with associated sinus pericranii and developmental venous anomaly. (a) Axial contrast-enhanced CT image shows a lobular intraconal mass on the left, which enhances as much as muscle. (b) Coronal contrast-enhanced CT image shows that some portions of the multilobular mass have enhancement features similar to those of muscle (arrowheads) and that another portion is less enhancing (arrow). (c) Coronal CT image shows abnormal veins along the walls of the lateral ventricles (arrowhead). (d) Coronal CT image (bone window) shows multiple bony defects of sinus pericranii in the left frontal bone (arrowhead) that allow anomalous venous drainage from the face to the intracranial venous structures.
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Figure 13b. Venous-lymphatic malformation of the orbit and face with associated sinus pericranii and developmental venous anomaly. (a) Axial contrast-enhanced CT image shows a lobular intraconal mass on the left, which enhances as much as muscle. (b) Coronal contrast-enhanced CT image shows that some portions of the multilobular mass have enhancement features similar to those of muscle (arrowheads) and that another portion is less enhancing (arrow). (c) Coronal CT image shows abnormal veins along the walls of the lateral ventricles (arrowhead). (d) Coronal CT image (bone window) shows multiple bony defects of sinus pericranii in the left frontal bone (arrowhead) that allow anomalous venous drainage from the face to the intracranial venous structures.
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Figure 13c. Venous-lymphatic malformation of the orbit and face with associated sinus pericranii and developmental venous anomaly. (a) Axial contrast-enhanced CT image shows a lobular intraconal mass on the left, which enhances as much as muscle. (b) Coronal contrast-enhanced CT image shows that some portions of the multilobular mass have enhancement features similar to those of muscle (arrowheads) and that another portion is less enhancing (arrow). (c) Coronal CT image shows abnormal veins along the walls of the lateral ventricles (arrowhead). (d) Coronal CT image (bone window) shows multiple bony defects of sinus pericranii in the left frontal bone (arrowhead) that allow anomalous venous drainage from the face to the intracranial venous structures.
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Figure 13d. Venous-lymphatic malformation of the orbit and face with associated sinus pericranii and developmental venous anomaly. (a) Axial contrast-enhanced CT image shows a lobular intraconal mass on the left, which enhances as much as muscle. (b) Coronal contrast-enhanced CT image shows that some portions of the multilobular mass have enhancement features similar to those of muscle (arrowheads) and that another portion is less enhancing (arrow). (c) Coronal CT image shows abnormal veins along the walls of the lateral ventricles (arrowhead). (d) Coronal CT image (bone window) shows multiple bony defects of sinus pericranii in the left frontal bone (arrowhead) that allow anomalous venous drainage from the face to the intracranial venous structures.
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Figure 14a. Dramatically enlarging venous-lymphatic malformation in a 9-year-old girl born with a left orbital mass (diagnosed at birth as a lymphangioma). (a) Axial T1-weighted image shows a multilobular mass with varied internal signal intensity and a fluid-fluid level (arrow). (b) Axial T2-weighted image shows the multilobular lesion with multiple fluid-fluid levels (arrowhead). (c) Axial proton-density–weighted image with fat saturation demonstrates the varied signal-intensity, multilobular mass with fluid-fluid levels (arrow).
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Figure 14b. Dramatically enlarging venous-lymphatic malformation in a 9-year-old girl born with a left orbital mass (diagnosed at birth as a lymphangioma). (a) Axial T1-weighted image shows a multilobular mass with varied internal signal intensity and a fluid-fluid level (arrow). (b) Axial T2-weighted image shows the multilobular lesion with multiple fluid-fluid levels (arrowhead). (c) Axial proton-density–weighted image with fat saturation demonstrates the varied signal-intensity, multilobular mass with fluid-fluid levels (arrow).
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Figure 14c. Dramatically enlarging venous-lymphatic malformation in a 9-year-old girl born with a left orbital mass (diagnosed at birth as a lymphangioma). (a) Axial T1-weighted image shows a multilobular mass with varied internal signal intensity and a fluid-fluid level (arrow). (b) Axial T2-weighted image shows the multilobular lesion with multiple fluid-fluid levels (arrowhead). (c) Axial proton-density–weighted image with fat saturation demonstrates the varied signal-intensity, multilobular mass with fluid-fluid levels (arrow).
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Figure 15a. Solitary form of juvenile fibromatosis in a 4-year old boy with painless swelling of the right lower eyelid. (a) Axial CT image (bone window) shows erosion of the lateral wall of the orbit and an adjacent soft-tissue mass (arrowhead). (b) Axial T1-weighted MR image demonstrates the well-circumscribed mass (arrowhead), which has signal intensity similar to that of muscle, invading the cortex of the adjacent bone. (c) Coronal contrast-enhanced T1-weighted image reveals the well-circumscribed mass with peripheral enhancement. (d) Photomicrograph (original magnification x200; H-E stain) of a specimen from a different patient with the same diagnosis reveals spindle cells with ellipsoid nuclei distributed in a storiform pattern of interlacing fascicles within a collagenous background.
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Figure 15b. Solitary form of juvenile fibromatosis in a 4-year old boy with painless swelling of the right lower eyelid. (a) Axial CT image (bone window) shows erosion of the lateral wall of the orbit and an adjacent soft-tissue mass (arrowhead). (b) Axial T1-weighted MR image demonstrates the well-circumscribed mass (arrowhead), which has signal intensity similar to that of muscle, invading the cortex of the adjacent bone. (c) Coronal contrast-enhanced T1-weighted image reveals the well-circumscribed mass with peripheral enhancement. (d) Photomicrograph (original magnification x200; H-E stain) of a specimen from a different patient with the same diagnosis reveals spindle cells with ellipsoid nuclei distributed in a storiform pattern of interlacing fascicles within a collagenous background.
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Figure 15c. Solitary form of juvenile fibromatosis in a 4-year old boy with painless swelling of the right lower eyelid. (a) Axial CT image (bone window) shows erosion of the lateral wall of the orbit and an adjacent soft-tissue mass (arrowhead). (b) Axial T1-weighted MR image demonstrates the well-circumscribed mass (arrowhead), which has signal intensity similar to that of muscle, invading the cortex of the adjacent bone. (c) Coronal contrast-enhanced T1-weighted image reveals the well-circumscribed mass with peripheral enhancement. (d) Photomicrograph (original magnification x200; H-E stain) of a specimen from a different patient with the same diagnosis reveals spindle cells with ellipsoid nuclei distributed in a storiform pattern of interlacing fascicles within a collagenous background.
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Figure 15d. Solitary form of juvenile fibromatosis in a 4-year old boy with painless swelling of the right lower eyelid. (a) Axial CT image (bone window) shows erosion of the lateral wall of the orbit and an adjacent soft-tissue mass (arrowhead). (b) Axial T1-weighted MR image demonstrates the well-circumscribed mass (arrowhead), which has signal intensity similar to that of muscle, invading the cortex of the adjacent bone. (c) Coronal contrast-enhanced T1-weighted image reveals the well-circumscribed mass with peripheral enhancement. (d) Photomicrograph (original magnification x200; H-E stain) of a specimen from a different patient with the same diagnosis reveals spindle cells with ellipsoid nuclei distributed in a storiform pattern of interlacing fascicles within a collagenous background.
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Figure 16a. Juvenile fibrosarcoma in a 5-year-old boy with right eye swelling, pain, and erythema. (a) Axial contrast-enhanced CT image shows a right intraconal, slightly circumscribed mass (arrowhead) that enhances as much as extraocular muscle. (b) Coronal T1-weighted image depicts the mass as distinct from and displacing the optic nerve (arrowhead). (c) Axial T1-weighted MR image shows that the mass is well defined and slightly hyperintense relative to muscle and brain. (d) Photomicrograph (original magnification x400; Masson trichrome stain) of a specimen from a different patient with the same diagnosis demonstrates spindle-shaped cells with elongate nuclei arrayed in a herringbone pattern of interlacing fascicles. Note the lack of significant collagenous matrix, which stains blue.
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Figure 16b. Juvenile fibrosarcoma in a 5-year-old boy with right eye swelling, pain, and erythema. (a) Axial contrast-enhanced CT image shows a right intraconal, slightly circumscribed mass (arrowhead) that enhances as much as extraocular muscle. (b) Coronal T1-weighted image depicts the mass as distinct from and displacing the optic nerve (arrowhead). (c) Axial T1-weighted MR image shows that the mass is well defined and slightly hyperintense relative to muscle and brain. (d) Photomicrograph (original magnification x400; Masson trichrome stain) of a specimen from a different patient with the same diagnosis demonstrates spindle-shaped cells with elongate nuclei arrayed in a herringbone pattern of interlacing fascicles. Note the lack of significant collagenous matrix, which stains blue.
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Figure 16c. Juvenile fibrosarcoma in a 5-year-old boy with right eye swelling, pain, and erythema. (a) Axial contrast-enhanced CT image shows a right intraconal, slightly circumscribed mass (arrowhead) that enhances as much as extraocular muscle. (b) Coronal T1-weighted image depicts the mass as distinct from and displacing the optic nerve (arrowhead). (c) Axial T1-weighted MR image shows that the mass is well defined and slightly hyperintense relative to muscle and brain. (d) Photomicrograph (original magnification x400; Masson trichrome stain) of a specimen from a different patient with the same diagnosis demonstrates spindle-shaped cells with elongate nuclei arrayed in a herringbone pattern of interlacing fascicles. Note the lack of significant collagenous matrix, which stains blue.
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Figure 16d. Juvenile fibrosarcoma in a 5-year-old boy with right eye swelling, pain, and erythema. (a) Axial contrast-enhanced CT image shows a right intraconal, slightly circumscribed mass (arrowhead) that enhances as much as extraocular muscle. (b) Coronal T1-weighted image depicts the mass as distinct from and displacing the optic nerve (arrowhead). (c) Axial T1-weighted MR image shows that the mass is well defined and slightly hyperintense relative to muscle and brain. (d) Photomicrograph (original magnification x400; Masson trichrome stain) of a specimen from a different patient with the same diagnosis demonstrates spindle-shaped cells with elongate nuclei arrayed in a herringbone pattern of interlacing fascicles. Note the lack of significant collagenous matrix, which stains blue.
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Figure 17a. Multifocal infantile fibromatosis in an 8-month-old girl with a 3-month history of epistaxis and medial deviation of the right eye. (a) Anteroposterior radiograph of the skull shows expansion of the sphenoid bone (arrow). (b) Coronal contrast-enhanced CT image (soft-tissue window) reveals an enhancing mass centered in the sphenoid sinus with a central unenhancing portion, findings consistent with necrosis or hemorrhage. (c) Anteroposterior radiograph of the femora shows lytic lesions with partially sclerotic margins in the left femoral neck and right distal femoral metaphysis (arrows).
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Figure 17b. Multifocal infantile fibromatosis in an 8-month-old girl with a 3-month history of epistaxis and medial deviation of the right eye. (a) Anteroposterior radiograph of the skull shows expansion of the sphenoid bone (arrow). (b) Coronal contrast-enhanced CT image (soft-tissue window) reveals an enhancing mass centered in the sphenoid sinus with a central unenhancing portion, findings consistent with necrosis or hemorrhage. (c) Anteroposterior radiograph of the femora shows lytic lesions with partially sclerotic margins in the left femoral neck and right distal femoral metaphysis (arrows).
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Figure 17c. Multifocal infantile fibromatosis in an 8-month-old girl with a 3-month history of epistaxis and medial deviation of the right eye. (a) Anteroposterior radiograph of the skull shows expansion of the sphenoid bone (arrow). (b) Coronal contrast-enhanced CT image (soft-tissue window) reveals an enhancing mass centered in the sphenoid sinus with a central unenhancing portion, findings consistent with necrosis or hemorrhage. (c) Anteroposterior radiograph of the femora shows lytic lesions with partially sclerotic margins in the left femoral neck and right distal femoral metaphysis (arrows).
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Copyright © 2007 by the Radiological Society of North America.
