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Sarcoma
Volume 7 (2003), Issue 2, Pages 87-91
http://dx.doi.org/10.1080/1357714031000081207
Case Report

Malignant Glomus Tumour: A Case Report and Review of the Literature

1Department of Pathology, Istituto dei Tumori di Napoli ‘G. Pascale’ di Napoli, Via M. Semmola, Napoli 80131, Italy
2Division of Medical Oncology B, Istituto dei Tumori di Napoli ‘G. Pascale’ di Napoli, Napoli, Italy
3Division of Surgical Oncology B, Istituto dei Tumori di Napoli ‘G. Pascale’ di Napoli, Napoli, Italy
4Division of Radiotherapy, Istituto dei Tumori di Napoli ‘G. Pascale’ di Napoli, Napoli, Italy
5Department of Pathology, Facoltà di Medicina e Chirurgia di Padova, Padova, Italy

Copyright © 2003 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose: Glomus tumours are characteristically benign solitary tumours. At our knowledge, about 23 reports are present in literature regarding the malignant counterpart, but only a minority developed metastases. We describe a locally aggressive glomus tumour with lymphnode metastasis.

Patient: The patient was a 40 year-old man presenting a 1.5-cm lesion on the right wrist incompletely excised and a recurrent tumour, 4 × 2 cm in size, removed after 9 months, for which he received radiotherapy. After 2 years he developed an axillary lymphnode metastasis.

Results: Histologically, both tumours (primary and metastasis) were similar. There were sheets and nests of uniform small cells with scant eosinophilic cytoplasm and round to polygonal nuclei; there was some degree of pleomorphism and the mitotic index was high (up to 18 m/10 HPF). The tumour cells were positive for vimentin and smooth muscle actin, but negative for desmin, NSE, Factor VIII, chromogranin, cytokeratin. Remarkably, in the primary, the cells strongly expressed p53 (70%) and MIB-1 (35%).

Discussions: In many reported malignant cases, the histology of the tumour cells suggested that they were malignant, yet the clinical course has been benign. Carefully reviewing the literature, it seems that actually we have enough histological criteria to identify the cases with biological adverse outcome. Those unfortunate cases behave as high grade sarcomas and therefore may deserve an aggressive therapeutic treatment.