Marrow involvement by metastatic spindle cell angiosarcoma

Wing Kit Lam and Sze Fai Yip

A 76-year-old woman presented with splenic hematoma with abdominal pain, hypovolemic shock, anemia, and thrombocytopenia (hemoglobin 6.3 g/dL, platelets 41 × 109/L). Urgent contrast computerized tomography of the abdomen showed a large splenic hematoma and a few hypodense lesions in the liver. Chest radiograph showed a 1.9-cm right upper lung mass, and positron emission tomography/computed tomography showed hypermetabolic lesions in lung, liver, spleen, intra-abdominal lymph nodes, and bone. Right upper lung transbronchial biopsy showed adenocarcinoma. Peripheral blood film was leukoerythroblastic. Right trephine biopsy showed focal infiltration by large spindle-shaped cells with large hyperchromatic nuclei and open chromatin with surrounding hemorrhages (panel A, original magnification ×200, hematoxylin and eosin [H&E] stain; panel B, original magnification ×400, H&E stain) and background megakaryocytic hyperplasia and fibrosis (panel C, original magnification ×200, H&E stain; panel D, original magnification ×200, reticulin stain). Immunohistochemistry showed that the tumor cells were positive for CD34, c-kit (CD117), CD31, smooth muscle α-actin (1A4), and erythroblastic transformation–specific related gene (ERG) (panels E-I, respectively, original magnification ×400) and negative for MNF116, cytokeratin (AE1/AE3), p63, desmin, S100, and mast cell tryptase.

Marrow involvement by metastatic spindle cell angiosarcoma is extremely rare. Sometimes, reactive megakaryocytic hyperplasia and fibrosis can lead to misinterpretation. Careful microscopic examination cannot be overemphasized. ERG is a highly specific endothelial marker that is useful in the diagnosis of this rare malignancy. Spleen is the most likely primary site in our patient, with a risk for spontaneous bleeding.


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