Abstract
A rare case of advanced neuroendocrine carcinoma of thigh in a young adult female, diagnosed with the aid of immunohistochemistry and electron microscopic examinations together with a review of the literature are reported. The primary tumor involved the lateral aspect of thigh without bone involvement. Patient showed significant response with adjuvant chemoradiotherapy following incomplete surgery.
Highlights
Neuroendocrine tumours (NET) are tumours arising from neuroendocrine cells or neurosecretory cells of neural crest origin
A 30 years young female presented to us in April 2011, with large diffuse swelling over lower end of left thigh which had been progressively increasing for 5 months
On examination she had a painless firm swelling involving the lateral aspect of left lower thigh of size about 15 cm × 10 cm. evident on 64 slice contrast enhanced CT scan, which showed a ill defined heterogeneously enhancing soft tissue lesion with nodularity involving the muscles of lateral compartment of lower end of left thigh with stranding and thickening of overlying subcutaneous fat plane and a small sclerotic lesion on condyle of left tibia without bone or knee joint involvement (Figure 1)
Summary
Neuroendocrine tumours (NET) are tumours arising from neuroendocrine cells or neurosecretory cells of neural crest origin. These are characterized by the presence of neurosecretory granules often producing biogenic amines and hormones [1]. Neuroendocrine tumors (NETs) are considered rare tumors, annual incidence of these tumors is approximately 6.5 - 5 per 500,000. Majority of them in which about 2/3rd are low grade carcinoid tumors and the rest 1/3rd account for other NETs [2]. Neuroendocrine tumors most commonly occur in the intestine, but may occur in the lung and other parts of the body [3,4]. Primary soft tissue NET is excessively rare, only few case studies are available in literature [5,6].
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