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Year : 2015  |  Volume : 52  |  Issue : 4  |  Page : 688--689

Intrascrotal paratesticular malignant fibrous histiocytoma-a rare case and its management

AA Kallianpur1, A Kapali1, NK Shukla1, SVS Deo1, D Muduly1, R Yadav2,  
1 Department of Surgical Oncology, All India Institute of Medical Science, New Delhi, India
2 Department of Pathology, All India Institute of Medical Science, New Delhi, India

Correspondence Address:
A A Kallianpur
Department of Surgical Oncology, All India Institute of Medical Science, New Delhi
India




How to cite this article:
Kallianpur A A, Kapali A, Shukla N K, Deo S, Muduly D, Yadav R. Intrascrotal paratesticular malignant fibrous histiocytoma-a rare case and its management.Indian J Cancer 2015;52:688-689


How to cite this URL:
Kallianpur A A, Kapali A, Shukla N K, Deo S, Muduly D, Yadav R. Intrascrotal paratesticular malignant fibrous histiocytoma-a rare case and its management. Indian J Cancer [serial online] 2015 [cited 2020 Oct 19 ];52:688-689
Available from: https://www.indianjcancer.com/text.asp?2015/52/4/688/178381


Full Text

Sir,

Paratesticular malignant fibrous histiocytoma (MFH) is an extremely rare malignancy of the scrotum, and scant information exists on the optimal management of this cancer.

We report a case of 71 year old male who presented with two months history of right testicular lump. In an outside hospital patient underwent right transcrotal orchidectomy in April 2009. Histopathology findings of the specimen were compatible with malignant mesenchymal tumor. Slides and blocks were reviewed in our hospital and revealed same morphology as the main specimen. Patient received six cycles of adriamycin and cyclophosphamide based adjuvant chemotherapy. Patient was disease free for 21 months before he developed local recurrence. On local examination there was a 10 × 8 cm lobulated mass at the right inguinal region involving the left testis with no overlying skin involvement. Contrast enhanced computerized tomography confirmed an 10 cm heterogenous enhanced mass at the root of penis and separate mass at the left testis. Fine needle aspiration of the lump was suspicious for MFH with immunocytochemistry positive for CD68. With the interim diagnosis of recurrent left paratesticular soft tissue sarcoma, the patient underwent wide local excision of the mass with total scrotectomy and left high level orchidectomy. Grossly a large single, multilobulated and nodular solid grey white mass replacing nearly the whole left testis and paratesticular tissue and extending to the inguinal region up to the deep ring [Figure 1]. The mass had spared the root of the penis. Microscopically the tumor consisted of highly pleomorphic spindle tumor cells arranged in a storiform pattern with numerous multinucleated giant cells and frequent mitoses (mitotic count of approx 10-12/10 hpf) along with areas of necrosis (less than 50% tumor necrosis for all the examined tumor surface) [Figure 2], [Figure 3]. According to the French Federation of Cancer Sarcoma Group, the neoplasm was diagnosed grade III sarcoma. Immunohistochemistry staining with CD 68 [Figure 4] and did not react with S100, NF, actin, desmin, smooth muscle actin and myogenin. The patient was planned for adjuvant external beam radiotherapy to the locoregional area.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Paratesticular sarcomas are an extremely rare entity and a paucity of information about their management exists in the literature. MFH is a highly aggressive tumor which commonly involves the extremities. MFH of the paratesticular region are much more rare malignancy with less than 43 reported cases till date.[1],[2] They usually arise from the testicular tunics or the spermatic cord.[3] Histopathologically is classified into four types: storiform-pleomorphic, myxoid, giant cell and inflammatory with storiform type being the most common. MFH may mimic the histopathologic appearance of other sarcomas, e.g., de-differentiated and pleomorphic liposarcoma, pleomorphic leiomyosarcoma. Pleomorphic sarcomas have a characteristically illdefined fascicular pattern, cellular and nuclear pleomorphism, bizarre tumor cells with frequent mitoses and necrosis. In this case the other sarcomas were ruled by the absence of any differentiation towards them (conventional/classical area of liposarcoma, leiomyosarcoma, rhabdomyosarcoma, nerve sheath tumor) in total examined tumor surface. The tumor showed similar morphology in all the areas and the specimen was extensively sampled. Still no area of adipocytic, smooth muscle, skeletal muscle or neural differentiation was seen. Moreover, negative immunohistochemical staining (S 100, NF, smooth muscle actin, desmin and myogenin) ruled out these sarcomas. MFH has no specific markers, but can show focal positivity for CD68, S100 and vimentin and is a diagnosis of exclusion.

MFH tends to recur locally thereby the treatment of choice is radical orchiectomy, with complete removal of all seminal cord structures. Ligation of the spermatic cord should be as high as possible. Despite complete resection, local recurrence has been reported to be 51%. Adjuvant locoregional (ipsilateral pelvic and groin nodes and scrotum) radiation reduces the risk of local recurrence.[4] Fagundes et al., reported no local recurrence in patients receiving adjuvant radiation, compared with 37% local failure in those treated with orchidectomy alone.[4] Modest activity with doxorubicin and dacarbazine based chemotherapy has been reported with MFH.[5],[6]

 Conclusion



Paratesticular MFH are uncommon tumors with a high tendency towards local recurrence. Despite the poor prognosis a prediction of the individual biological behavior of the locally recurrent tumor is difficult. Local wide excision and adjuvant radiochemotherapy might enable long-term survival in selected cases.

References

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2Takezawa K, Matsuoka Y, Takao T, Nonomura N, Tomita Y, Okuyama A. Myxofibrosarcoma of the spermatic cord: A case report. Nihon Hinyokika Gakkai Zasshi 2008;99:555-9.
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