Rare and aggressive metastatic, axial multifocal local epithelioid sarcoma associated with paraneoplastic granulocytosis and hypoglycaemia

Epithelioid sarcoma is a rare soft-tissue sarcoma (STS; <1% of total STS) that aff ects young, usually male, adults or adolescents. In most cases, the sarcoma grows slowly (over a period of years), involves the dermis, subcutis, or deeper soft tissues in the distal extremities in 55–60% of cases. Epithelioid sarcoma is characterised by diagnostic diffi culties, both clinically and histopathologically, which results in a high frequency of initial misdiagnosis and loss of crucial treatment time. Surgical excision (amputation or wide en-bloc excision), then high-dose radiotherapy represents optimum treatment, however, this regimen is associated with a recurrence rate of 77% and a median overall survival of about 88 months for patients without distant metastases, and just 8 months for those with distant metastases. Unlike in most sarcomas, metastases aff ecting the lymph nodes are common in epithelioid sarcoma. However, the lungs are the most common site of distant metastases in epithelioid sarcoma. Furthermore, multifocal local disease, initial proximal limb or axial tumour location, regional metastases, high mitotic fi gures, necrosis, and haemorrhage or vascular invasion have a more aggressive course, and predict for a poor survival that is free from distant metastasis. Immunohistochemically, most epithelioid sarcoma express vimentin, keratin, epithelial membrane antigen (EMA), CD34 (60%), cyclin D1 and tissue polypeptide antigen (TPA). Coexpression of vimentin and keratin is thought to be characteristic of epithelioid sarcoma. A 37-year-old man was admitted to our hospital diagnosed with epithelioid sarcoma. On admission, the patient had four cutaneous lesions measuring 3–6 cm and a painful right knee. The lesions were localised at the epigastrium, right lateral abdominal area, anterior iliac crest level, spinal region at the level of L3–L5, and right buttock. According to the patient’s recent medical history, the fi rst lesion of around 1 cm seemed to develop on a surgical scar 3 months after a gastric ulcer operation. The lesion was misdiagnosed as infl ammation and was treated with antibiotics. 1 month later, a second lesion developed on his right buttock, and a biopsy by surgical excision yielded a diagnosis of epithelioid sarcoma. Microscopic examination shows nodular growth with central necrosis and haemorrhage (fi gure 1A). Immunohistochemistry showed expression of vimentin (fi gure 1B) and actin. EMA, keratin, CEA, S100p, CD30, CD31, and CD34 were negative. CT scans of the thorax and abdomen, and a bone scan found splenomegaly, multiple lung nodules, enlarged mediastinal, mesenteric, and paraortic lymph nodes, and bone metastases at L3, L5 vertebrae and at the right upper tibia (fi gure 2). Blood analyses showed leucocytosis (white blood cell [WBC] count: 23·9 × 10 cells/L; with neutrophils: 87·9%). Multiple cultures of blood, urine, sputum, and dermal lesions found no evidence of infection during the patient’s clinical course. Based on prominent leucocytosis, we measured serum granulocyte colony-stimulating factor (G-CSF). The highly increased serum G-CSF of more than 2 μg/L (normal <40 ng/L) suggested that a G-CSF-producing epithelioid sarcoma was the cause of leucocytosis. The Lancet Oncol 2006; 7: 82–84