MyMedR

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.

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*

Fulltext Primary hepatic angiosarcoma: difficulty in clinical, radiological, and pathological diagnosis

Yang KF, Leow VM, Hasnan MN, Subramaniam MK

Med J Malaysia, 2012 Feb;67(1):127-8.

PMID: 22582567 MyJurnal

Hepatic angiosarcoma is a rare primary mesenchymal malignancy. Prognosis is poor and mortality occurs early. The diagnosis is challenging. Our case was an asymptomatic 70 year-old man referred, with incidental ultrasonography finding of multiple liver nodules. Diagnostic laparoscopic liver biopsy and the histopathological examination reported a haemangioma. Six months later, he became symptomatic and his health condition deteriorated rapidly.

Matched MeSH terms: Hemangiosarcoma/diagnosis*

Hemangioendotheliosarcoma

Dutt AK

Med J Malaya, 1969 Dec;24(2):161-3.

PMID: 4244145

Matched MeSH terms: Hemangiosarcoma*

Fulltext Haemorrhaging lesion in the breast: is there a role for embolisation?

Taib N, Yip Ch, Ranganathan S, Moosa F, Mun K

Biomed Imaging Interv J, 2006 Jul;2(3):e30.

PMID: 21614243 MyJurnal DOI: 10.2349/biij.2.3.e30

Angiosarcoma of the breast is an extremely rare condition. This case illustrates the use of embolisation as a modality of treatment for primary breast angiosarcoma. No other case has been reported on the use of embolisation for this disorder.

Matched MeSH terms: Hemangiosarcoma

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/complications; Hemangiosarcoma/drug therapy; Hemangiosarcoma/pathology*; Hemangiosarcoma/radiotherapy; Hemangiosarcoma/surgery

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*; Hemangiosarcoma/surgery

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/diagnosis*; Hemangiosarcoma/pathology*

Fulltext Concurrent Double Primary Tumours: Papillary Thyroid Carcinoma and Cervical Epithelioid Angiosarcoma

Lum SG, Mahmud N, Isa N, Mat Baki M

Medeni Med J, 2020;35(4):349-355.

PMID: 33717629 DOI: 10.5222/MMJ.2020.83479

Angiosarcoma is one of the rarest types of malignant vascular tumours that involved the head and neck region. It predominantly affects the scalp and superficial soft tissues. Angiosarcoma arising from the deep cervical soft tissue is extremely rare. There is a limited literature on neck angiosarcoma that occurred simultaneously with papillary thyroid carcinoma. We report a rare case of concurrent papillary thyroid carcinoma and cervical epithelioid angiosarcoma, and postoperative rapid progression of residual angiosarcoma that mimicked a neck haematoma. The diagnostic challenge and possible etiologies have been discussed here.

Matched MeSH terms: Hemangiosarcoma

Nasopharyngeal undifferentiated carcinoma with sarcomatoid features: Pitfalls in the immunohistochemistry

Lee M, Son HJ, Kim NY, Kim SJ, Yu IK

Malays J Pathol, 2019 Aug;41(2):201-206.

PMID: 31427557

We present a case of an undifferentiated subtype of non-keratinizing squamous cell carcinoma (NK-SCC) with sarcomatoid features in the nasopharynx in a 69-year-old man who was difficult to diagnose due to spindle-shaped malignant cells. He was admitted because of a right nasal obstruction and right headache, and imaging revealed a heterogeneously enhanced irregularly shaped mass at the nasopharynx. Histopathologically, the tumour was partially organised, and the tumour cells were epithelioid or spindle-shaped. Initially, we erroneously diagnosed the tumour as an angiosarcoma owing to its false-negative immunoreaction for cytokeratins and a mistaken interpretation for CD31. After in situ hybridization for Epstein-Barr virus was positive, a consultation and additional immunostaining (including re-staining for cytokeratin with varying dilutions) were performed, and the diagnosis was revised to NK-SCC with sarcomatoid features. We believe that sarcomatoid features may be observed in nasopharyngeal carcinoma and in this case, immunostaining using various epithelial markers is necessary and careful attention should be paid to the interpretation of immunostaining.

Matched MeSH terms: Hemangiosarcoma

Intracranial neoplasms in Malaysia

Selby R, Pereira N

Int Surg, 1973 Aug;58(8):536-41.

PMID: 4738062

Matched MeSH terms: Hemangiosarcoma/epidemiology

Angiosarcoma - a rare fatal cause of recurrent pericardial effusions

Ng BH, Tan YS, Pavitratha P, A Hing C, Zainul NH, Lim CH

Med J Malaysia, 2020 11;75(6):759-761.

PMID: 33219196

A 40-year-old man presented to the Hospital Sultanah Bahiyah, Alor Setar, Kedah, with constitutional and respiratory symptoms. Physical examination and echocardiogram demonstrated massive pericardial effusion. Patient required multiple attempts of pericardiocentesis due to recurrent pericardial effusion. Initial workup including pericardial fluids examination and computed tomography imaging did not reveal any apparent cause. Magnetic resonance imaging showed a suspicious mass infiltrating into the right atrium. Autoimmune screening was negative. Patient was subsequently treated as having tuberculous pericarditis. However, his disease progressed rapidly and he eventually passed away due to right atrial rupture. Postmortem revealed a ruptured right atrial tumour leading to massive haemothorax. Histopathological examination confirmed the diagnosis of primary pericardial angiosarcoma.

Matched MeSH terms: Hemangiosarcoma

Concurrent bariatric surgery and surgical resection of massive localized lymphedema of the thigh. A case report

Hou LG, Prabakaran A, Rajan R, Mohd Nor FB, Ritza Kosai N

Ann Med Surg (Lond), 2019 Nov;47:53-56.

PMID: 31687133 DOI: 10.1016/j.amsu.2019.10.002

Introduction: Massive localised lymphedema (MLL) is considered a rare large, pendulous localised benign overgrowth of lymphoproliferative tissue commonly seen in patients with morbid obesity. Histologically, it may be mistaken for well-differentiated liposarcoma; hence, it is also known as pseudosarcoma.

Presentation of case: We describe the successful management of MLL of the left medial thigh in a 35-year-old man weighing 220 kgs (BMI 80.8 kgs/m2). He underwent a concurrent laparoscopic sleeve gastrectomy with surgical resection of the MLL. He recovered well and during our last follow up six months after the operation, he is ambulating well and weighs 148 kgs (BMI 54.4 kgs/m2).

Discussion: MLL is a form of secondary lymphedema resulting in disruption or compression of normal lymphatic drainage due to fat accumulation in obese patients. Patients usually delay treatment for even up to a decade, when it becomes sufficiently large enough to restrict mobility and daily activities, or when it becomes infected. MLL is primarily a clinical diagnosis. A detailed history regarding its slow growth spanning over the years makes malignancy less likely. However, if left untreated, MLL may progress to angiosarcoma. Imaging studies such as computed tomography (CT) and a Magnetic Resonance Imaging (MRI) are usually performed to rule out malignancy or vascular malformations. A tissue biopsy is not recommended unless there are suspicious pigmented lesions.

Conclusion: MLL remains to be underdiagnosed. Due to the obesity epidemic, clinicians must be aware of this once rare disease. The role of concurrent bariatric surgery with surgical resection of MLL warrants further studies.

Matched MeSH terms: Hemangiosarcoma

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