Primary Orbital Angiosarcoma

Siddens, John D.; Fishman, Jefrey R.A.; Jackson, Ian T.; Nesi, Frank A.; Tsao, Kailenn

doi:10.1097/00002341-199911000-00019

Cited by 13 publications

(3 citation statements)

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“…This is in contrast with the deeper location of other soft tissue sarcomas. Less frequently it can occur in various organs and has been reported in uterus [1], ovary [2], small intestine [3], lung [4], heart [5], oral cavity [6], orbit [7] and thyroid [8]. Unlike angiosarcomas of the skin, deep angiosarcomas more commonly have an epitheloid appearance consisting of nests and clusters of round cells of high nuclear grade.…”

Section: Introductionmentioning

confidence: 99%

High grade angiosarcoma arising in fibroadenoma

et al. 2011

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Primary angiosarcoma of the breast is a rare tumour that account for fewer than 0.05% of all malignant mammary tumours. Angiosarcoma may have an perfidious clinical onset. Radiologic findings are often nonspecific and may appear completely normal in one-third of cases with primary angiosarcoma. The prognosis is usually poor because of the high rates of local recurrence and early development of metastases. Aggressive surgical resection is the mainstay of treatment. The role of adjuvant therapy has not yet been well established.Here we present a case of a 53 year old, postmenopausal women with primary angiosarcoma arising in fibroadenoma. To our knowledge, this is the first case described in the literature to date.

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Section: Introductionmentioning

confidence: 99%

High grade angiosarcoma arising in fibroadenoma

et al. 2011

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“…The tumor usually presents patches, nodules, and ulcers. There are reports of similar angiosarcomas presenting eyelid swelling from dermatology but rarely from ophthalmology [12-15]. On comparing the clinical findings of the eyelids, the left eyelid of case 1 appeared hard on palpation at initial presentation suggesting direct tumor invasion.…”

Section: Discussionmentioning

confidence: 94%

Two Cases of Angiosarcoma with Persistent Unilateral Eyelid Swelling

Sakisaka

Tanabe

Imayama³

et al. 2021

Ocul Oncol Pathol

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Angiosarcoma is a widely known neoplasm with one of the poorest prognoses; however, such cases are rarely observed by the ophthalmologists in clinical practice. The tumor commonly develops on the face and scalp and may cause eyelid swelling. We present two such cases. Case 1 was an 82-year-old woman who presented with indulated swelling of the right eyelid that extended to the upper forehead. Biopsy revealed a poorly circumscribed tumor infiltrated throughout the dermis and fat tissue, exhibiting both angiomatous and solid patterns. Immunohistochemistry showed CD31 (-) and D2-40/podoplanin(+), suggesting angiosarcoma. The patient had multiple lung metastases and died 14 months later. Case 2 was a 77-year-old man who presented with swelling of the left eyelid and erythema on the left temporal skin developed ulcerated nodules. Biopsy showed proliferated tumor cells in a sheet form, extensively throughout the dermis. Angiosarcoma was preferred based on immunohistochemistry: CD31(+), CD34(+), D2-40(-). The patient was treated with electron beam therapy and chemotherapy, and died after 13 months. It would be difficult for an ophthalmologist to suspect such a rare neoplasm only from swelling of the eyelids. However, angiosarcoma should be considered if the swelling spreads steadily from a head rash with a nodule or ulcer nearby.

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“…[3][4][5] Angiosarcoma of the eyelid has been reported before, and indeed the patients who could be treated with radical surgical excision seemed to have a very good prognosis. [9][10][11][12] In our patient, there has been no sign of local recurrence or distant metastasis during 3 years of followup, albeit that the latter may still develop.…”

Section: Letter To the Journalmentioning

confidence: 94%