Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma is a rare, low grade vascular (endothelial) neoplasm typically presenting as multicentric, superficial to deep nodules in extremities with a slight tendency of affecting young adult males. We report a case of pseudomyogenic hemangioendothelioma in a 15-year-old boy presenting initially with a 1 cm right thigh painless cutaneous lump. The lump was excised with the clinical impression of a sebaceous cyst. On microscopy, a poorly circumscribed, mild to moderately atypical spindle cell lesion in fascicular and storiform patterns with strikingly myoid-like eosinophilic cytoplasm was identified. The spindle cells were highlighted by pancytokeratin AE1/AE3, CD31, and ERG with retained INI-1, while being negative for MNF116, S100, CD34, EMA, desmin, SMA, caldesmon, myogenin, MyoD1, HHV-8 and CD163. Following the first diagnostic report, a positron emission tomography-computed tomography (PET-CT) scan revealed another 4 cm ill-defined nodule accompanied by a smaller adjacent 0.7 cm ipsilateral satellite nodule within the right psoas muscle that displayed similar morphology and immunophenotype as the cutaneous lump, supporting the multicentric feature of this unique entity. It is an uncommon yet increasingly recognised neoplasm of endothelial origin possessing a misleading myoid morphology and distinctive immunophenotype worth notifying.
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.
Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed and showed partial response to treatment for cardiac failure. A persistent right upper zone consolidation on chest radiograph prompted further investigations which revealed the diagnosis of pulmonary epithelioid hemangioendothelioma. The second patient presented with right-sided hemiparesis for 1-month duration. Initial computer tomography scan of the brain showed findings of distant metastatic foci. Subsequent investigations revealed pulmonary epithelioid hemangioendothelioma as the primary lesion. Both patients succumbed without any treatment due to rapid progression of the disease. We believe that pulmonary epithelioid hemangioendothelioma is undoubtedly rarely reported in south-east Asia region. In these two case reports, the patients were diagnosed in west and east Malaysia, respectively, in the same year (2015). Both cases highlight the increasing prevalence of pulmonary epithelioid hemangioendothelioma. We postulate that this could possibly be secondary to the advancement in diagnostic capabilities and improved healthcare facilities available in this region. Late presentation of pulmonary epithelioid hemangioendothelioma generally results in grave prognosis. Further investigations are required to elucidate the nature of progression and therapeutic options for patients with pulmonary epithelioid hemangioendothelioma.