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Review
. 2016 Feb 3;14(1):29.
doi: 10.1186/s12957-016-0789-5.

Epithelioid angiosarcoma arising in schwannoma of the kidney: report of the first case and review of the literature

G Iannaci  1 , M Crispino  2 , P Cifarelli  2 , M Montella  3 , I Panarese  3 , A Ronchi  3 , R Russo  3 , G Tremiterra  3 , R Luise  3 , P Sapere  4
Affiliations
Review

Epithelioid angiosarcoma arising in schwannoma of the kidney: report of the first case and review of the literature

G Iannaci et al. World J Surg Oncol. .

Abstract

Background: Schwannoma and angiosarcoma are infrequent pathologies that have been rarely reported in the kidney. Angiosarcoma is an uncommon malignant tumor presenting a recognizable vascular differentiation. It can develop in any site but the most common locations include the skin, soft tissues, breast, bone, liver, and spleen while renal localization has been very rarely reported in the literature. Schwannoma is a benign peripheral nerve sheath tumor composed of cells with the immunophenotype and ultrastructural features of differentiated Schwann cells. It has a wide anatomical distribution but the most frequent locations include subcutaneous tissues of the extremities and the head and neck region and the retroperitoneal and mediastinal soft tissues. The occurrence of an angiosarcoma in a pre-existing schwannoma is an extremely rare event with <20 cases reported in worldwide literature. In the present study, a renal case of angiosarcoma arising in schwannoma is presented with a detailed review of the pertinent literature.

Case presentation: A 56-year-old man was admitted with a few days history of lower back pain and hematuria. Abdominal ultrasound showed a mass inside the left renal medulla. Subsequent imaging investigations with computed tomography and magnetic resonance confirmed the presence of the lesion and showed a pulmonary metastasis.

Conclusions: The final histopathological examination led to the diagnosis of epithelioid angiosarcoma arising in a schwannoma. The patient came to death a few months later due to a massive hemothorax. To the best of our knowledge, the present is the first case of an angiosarcoma arising in a schwannoma of the kidney.

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Figures

Fig. 1
Fig. 1
a Computed tomography: imaging identifies an intraparenchymal nodule in the left lung. b Computed tomography: imaging identifies the presence of a nodular mesonephric hypodense lesion characterized by post contrastographic enhancement. c Gross imaging: the kidney shows an irregular profile with granulating appearance of the hilum. (d) Gross imaging: sectioned tumor appears hemorrhagic, soft, and reddish
Fig. 2
Fig. 2
a H&E staining (×10): numerous neoplastic vascular channels (left) that infiltrates the renal parenchyma (right). b H&E staining (×20): neoplastic cells forming vascular structures with epithelioid features and highly atypical nuclei. c H&E staining (×10): malignant cells of angiosarcoma (left) juxtaposed to benign spindle cells population of schwannoma (right). d H&E staining (×40): fine needle aspiration biopsy of the pulmonary lesion shows neoplastic cells
Fig. 3
Fig. 3
a, b Immunohistochemical staining (×10): neoplastic cells show immunopositivity for CD34 and cytokeratin. c Immunohistochemical staining (×10): schwannoma cells with strong, diffuse S100 immunoreactivity. d Immunohistochemical staining (×40): neoplastic cells present in fine-needle aspiration biopsy of the pulmonary lesion are CD34 positive
See this image and copyright information in PMC

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