Vulvar squamous cell carcinoma with sarcoma-like stroma: a case report and review of the literature

Abstract

Vulvar squamous cell carcinoma with sarcoma-like stroma represents an extremely rare histological entity showing the co-existence of both epithelial and mesenchymal features: these tumors, firstly described in the skin by Martin and Stewart in 1935 have been further described in other anatomic sites including oral cavity, larynx, breast, lung and oesophagus. The complexity of the histology, as well as its aggressive clinical behaviour makes the diagnosis and the exploitment of effective therapeutic approaches very difficult, so that no definitive guidelines for treatments are currently available. Here, we describe a case of advanced stage vulvar squamous cell carcinoma with sarcoma-like stroma showing an unfavourable prognosis despite the use of an aggressive multimodal approach. A revision of the currently published cases have been also provided.

Figure 1

Vulvar squamous cell carcinoma with sarcoma-like stroma (A, B, C, D, E tumor features at presentation site; F local tumor recurrence). A, B. Good squamous differentiation with keratinous pearls at tumor surface (upper-left corner in A and B) and less differentiated, spindle shaped cells in deeper portions of the tumor (right in A and B). Presence of scattered anaplastic, pseudosarcomatous cancer cells in a dense reactive stroma is shown in B (hematoxylin and eosin stain). C. At higher magnification ill defined cell borders are better seen (hematoxylin and eosin stain). D. Tumor cells presented positive for cytokeratins, more in the superficial, better differentiated portion of the tumor (upper-left corner). Inset shows the presence of reactivity for Cytokeratin in the isolated, giant neoplastic cells (Monoclonal Mouse Anti-Human Cytokeratin High Molecular Weight, clone 34βE12, DAKO, Carpinteria, CA, USA). E. Vimentin stain highlights a thick reactive vascular stroma, whereas giant and spindle shaped tumor cells appear negative (central portion of picture) (Dako, Carpinteria, CA, USA). F. Local tumor recurrence. Note the higher degree of tumor differentiation with abundance of keratinous pearls in this representative histological field (hematoxylin and eosin stain). Bars are 100 μm in A, B, D, F; 50 μm in E and D inset; 25 μm C.