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Primary uterine angiosarcoma with "rhabdoid morphology": A case report

Gandhi JS, Kamboj M, Gupta G, Seth N

Malays J Pathol, 2018 Aug;40(2):203-207.
PMID: 30173240

INTRODUCTION: Primary uterine angiosarcoma is a very rare tumour, with only 23 cases described till now. It is a malignant tumour with cells variably recapitulating the morphologic features of an endothelium and expressing immunohistochemical markers of endothelial cells. In general, it is a bulky neoplasm and frequently is at advance stage of disease at presentation. In general, patients with uterine angiosarcoma tend to have a poorer prognosis, mostly related to the aggressive nature and the metastatic potential of these tumours.
CASE REPORT: We report a rare case of primary uterine angiosarcoma with unusual rhabdoid morphology in a 41-year-old female, who underwent radical hysterectomy and died of disease after 4 months of treatment.
DISCUSSION: We described the differential diagnosis of primary angiosarcoma of the uterus that can pose a diagnostic challenge.

Matched MeSH terms: Hemangiosarcoma/pathology*
Angiosarcoma of the breast complicating pregnancy

Kaur G

Malays J Pathol, 1998 Jun;20(1):41-4.
PMID: 10879263

A 32-year-old pregnant lady presented with a rapidly enlarging right breast mass. A fine needle aspiration was suggestive of a malignant phylloides tumour. However histopathological examination after mastectomy revealed a moderately differentiated angiosarcoma. The histopathological and cytological features of this rare tumour together with the diagnostic pitfalls are discussed.

Matched MeSH terms: Hemangiosarcoma/pathology*
Primary angiosarcoma of the thyroid gland with recurrence diagnosed by fine needle aspiration: a case report

Isa NM, James DT, Saw TH, Pennisi R, Gough I

Diagn Cytopathol, 2009 Jun;37(6):427-32.
PMID: 19306411 DOI: 10.1002/dc.21065

Angiosarcoma of the thyroid is a rare and aggressive primary malignant tumor of the thyroid originally reported in patients from the Swiss Alpine region. Diagnosis of this tumor rests mainly on characteristic histopathological features of a malignant vascular tumor supported by immunopositivity for vascular markers e.g., CD31, Factor VIII, and CD34. Its cytological features, however, are not well-defined. We describe a case of primary angiosarcoma of the thyroid in a 48-year-old female, who presented with a rapidly enlarging neck mass associated with compressive symptoms. She had a history of hypothyroidism. The initial fine needle aspiration cytology of the neck mass was negative. She then underwent left hemithyroidectomy. Histologically, the tumor showed poorly differentiated malignant cells with eccentrically-placed nuclei, prominent nucleoli, and intracytoplasmic vacuoles admixed with mixed inflammatory cells. These showed immunopositivity for CD31 but were negative for CD34, Factor VIII, CK5/6, EMA, TTF-1, Thyroglobulin, Calcitonin, Melan A, and Calretinin. A diagnosis of poorly differentiated malignant tumor consistent with angiosarcoma was made. The patient was treated with radiation therapy but developed recurrence of the tumor. Second aspiration cytology of the recurrent tumor yielded hypocellular smears containing singularly dispersed atypical cells having eccentrically-placed nuclei with prominent macronucleoli and intracytoplasmic vacuoles within a background of inflammatory cells, consistent with recurrent angiosarcoma. Chemotherapy was started but she succumbed to the disease 7 months after diagnosis. The cytological, histopathological, immunohistochemical findings, and the clinical course are discussed.

Matched MeSH terms: Hemangiosarcoma/pathology*
Intramedullary spindle cell hemangioendothelioma of the thoracic spinal cord--case report

Abdullah JM, Mutum SS, Nasuha NA, Biswal BM, Ariff AR

Neurol. Med. Chir. (Tokyo), 2002 Jun;42(6):259-63.
PMID: 12116532

A 28-year-old Malay man presented with progressive paraparesis over a period of 6 months. Magnetic resonance imaging of the spine revealed a thoracic intramedullary spinal cord tumor at the T-7 level with homogeneous enhancement following intravenous gadolinium administration. Laminectomy and partial decompression of the tumor was performed. Histological examination of the tumor revealed features of spindle cell hemangioendothelioma. The patient was managed with limited field radiotherapy followed by systemic interferon therapy. Good neurological improvement was seen subsequently. The patient has survived 48 months with growth restraint at the primary site, although residual neurological deficit persists. Immunotherapy should be considered as a treatment modality for intramedullary hemangioendothelioma of the spinal cord after surgery and radiotherapy.

Matched MeSH terms: Hemangiosarcoma/pathology*