Teaching casesComplex karyotype in a case of cutaneous lymphangiosarcoma associated with chronic lymphedema of the lower limb
Introduction
Cutaneous lymphangiosarcoma is a rare malignant neoplasm of endothelial cells that accounts for less than 1% of all sarcomas [4]. A reliable distinction between lymphangiosarcoma and sarcoma with blood vascular differentiation is currently not feasible, however, lymphangiosarcoma is the term applied to an angiosarcoma associated with chronic lymphedema [4], [19]. This latter entity has been more frequently described in association with chronic lymphedema of the upper limb after mastectomy and radiotherapy for breast carcinoma [8], [23], [24]. In addition, it can arise in chronic, congenital or acquired, lymphedema of the lower limb [1]. These forms are considered as secondary angiosarcomas in contrast with de novo or primary forms.
Lymphangiosarcoma usually arises in elderly individuals and the prognosis is generally poor, with only 15% of patients being alive five years after diagnosis and treatment [19].
In order to differentiate lymphangiosarcoma from hemangiosarcoma, a number of lymphatic markers for immunohistochemistry have been recently proposed [13]. These include antibodies directed against Prox-1, vascular endothelial growth factor-3 (VEGFR-3), lymphatic vessel hyaluronan receptor-1 (LYVE-1) and podoplanin (recognized by the D2-40 clone). In particular, in the distinction between lymphatic and hematic endothelia podoplanin showed high specificity [2], [11], [13]. Furthermore, Mankey et al. [13] observed an association between D2-40 immunoreactivity and a hobnail pattern of malignant cells, suggesting that the concept of lymphangiosarcoma, originally based upon clinical findings, may evolve into a pathological definition based on morphological ground.
Here we report the morphological and immunohistochemical features of a rare case of cutaneous angiosarcoma of the leg. In addition, we performed on this case both a conventional and a molecular cytogenetic analysis and compared our results with a review of the previously reported data.
Section snippets
Histopathology and immunohistochemistry
We fixed tumor samples in buffered formalin (formaldehyde 4% w/v and acetate buffer 0.05 M) and routinely processed them to paraffin wax. Serial sections were stained with hematoxylin and eosin (H&E) for the histopathological evaluation.
For immunohistochemistry, 3 μm-thick sections were mounted on poly-L-lysine coated slides, deparaffinized, followed by the avidin–biotin complex (ABC) procedure according to Hsu et al. [9]. Immunoreactions were counterstained with Harris’ hematoxylin. The
Clinical case report
An 87-year-old woman, with a history of hypertension, phlebitis of the lower limbs, recurring lymphangitis and a pelvic fracture, presented with an ulcerated nodular lesion of the right leg, with a greatest diameter equal to 1.3 cm, which was biopsied and diagnosed as angiosarcoma. After a few days, a secondary lesion appeared on the skin of the right thigh, associated with a septic state, so the patient was treated with amputation of the lower limb; she did not receive chemotherapy or
Discussion
We reported a rare case of cutaneous lymphangiosarcoma of the leg, including morphological and cytogenetic features. The tumor arose in a patient with a long dated history of chronic lymphedema, confirming that this condition is a risk factor for the development of lymphangiosarcoma [1], [22], [23], [29]. The histopathological and immunohistochemical profile of the neoplastic cells were fully consistent with those of an angiosarcoma. The lack of HHV-8 ruled out a Kaposi's sarcoma. The strongly
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