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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 3  |  Issue : 3  |  Page : 235-237

An autopsy study of synchronous intratesticular and mediastinal sarcoma with pulmonary metastasis


Department of Pathology, Grant Government Medical College, Mumbai, Maharashtra, India

Date of Web Publication8-May-2014

Correspondence Address:
Sameer A. H. Ansari
Assistant Professor, Department of Pathology, Grant Government Medical College, Jamshedjee Jeejeebhoy Hospital, Byculla - 400 008, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.132118

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  Abstract 

Most testicular sarcomas arise from germ cell tumors (GCTs) viz. teratoma or spermatocytic seminoma. Pure intratesticular sarcomas are very rare. We present an autopsy report of non-germ cell primary intratesticular sarcoma with synchronous mediastinal sarcoma and lung metastasis in a young male who presented with symptoms of mediastinal compression and simultaneously had a significant gradually enlarging testicular mass.

Keywords: Intratesticular, lung metastasis, mediastinum, sarcoma


How to cite this article:
Momin YA, Ansari SA. An autopsy study of synchronous intratesticular and mediastinal sarcoma with pulmonary metastasis. Clin Cancer Investig J 2014;3:235-7

How to cite this URL:
Momin YA, Ansari SA. An autopsy study of synchronous intratesticular and mediastinal sarcoma with pulmonary metastasis. Clin Cancer Investig J [serial online] 2014 [cited 2019 Oct 23];3:235-7. Available from: http://www.ccij-online.org/text.asp?2014/3/3/235/132118


  Introduction Top


Intrascrotal sarcomas are divided into testicular and paratesticular sarcomas. Attempts have to be made to delineate the specific site of origin of the sarcoma. Since these structures are in continuity with each other it is not always possible to determine the exact origin in such infiltrative tumors. [1],[2] Paratesticular sarcomas refer to tumors which involve epididymis, scrotal wall, and spermatic cord. Intratesticular sarcomas are far less common, and comprise less than 1%. Majority of them, in reality are an element of germ cell neoplasia. [1],[2],[3] Pure intratesticular non-germ cell sarcomas are extremely rare with very few cases reported in the literature. [1],[2],[3],[4],[5],[6],[7],[8] To our knowledge, synchronous pure intratesticular and medistinal sarcoma has not been reported as yet.


  Case report Top


A 38-year-old male was admitted for progressive breathlessness, cough with mucoid expectoration, and intermittent chest pain for past 6 months, with a history of waxing and waning fever for 15 days. His physical examination revealed an enlarged left testis. The patient did not disclose about the testicular mass because it was painless, gradually progressing over a year. Chest X-ray and computed tomography (CT) chest showed right sided pleural effusion with a pleura-based mediastinal mass measuring 12 cm × 8 cm × 4 cm. Cytological study of pleural fluid was positive for malignant cells. Lung was biopsied, but the biopsy was non-representative. He succumbed on the 6 th day of hospitalization. Autopsy examination revealed 20 cc of turbid hemorrhagic fluid in the thoracic cavity. There was a large, irregular, gray tan left-sided mediastinal mass measuring 12 cm × 10 cm × 8 cm [Figure 1]. Cut sections of the mass showed fleshy appearance. The adhered lower lobe of left lung showed a distinct nodule measuring 2 cm × 2 cm. Other lung appeared unremarkable. Left testicle was enlarged with a bosselated appearance measuring 12 cm × 12 cm × 7 cm. Cut section showed a tumor mass with nodules showing areas of hemorrhage and extensive necrosis [Figure 2]. Contralateral testis was normal. There was no iliac lymphadenopathy. Histopathological examination of testicular mass showed a tumor composed of spindle cells arranged in a fascicular pattern [Figure 3]a, hemangiopericytomatous pattern [Figure 3]b, myxoid areas with cells arranged in reticular pattern [Figure 3]c, and focal chondroid area [Figure 3]d. Interspersed pleomorphic bizarre tumor giant cells [Figure 4], extensive necrotic areas, and brisk mitoses were evident. Thorough sampling and extensive search did not show any teratomatous or seminomatous element to exclude the germ cell origin of the tumor. Histopathological sections from the mediastinal mass and lung nodule showed exactly similar morphology like testicular tumor with fascicular arrangement of spindle cells, myxoid areas, and reticular pattern along with bizzare giant cells but devoid of cartilaginous foci. A panel of imunohistochemistry (IHC) including vimentin, cytokeratin, desmin, myogenin, smooth muscle actin (SMA), placental alkaline phosphatase (PLAP), S100, c-Kit, and CD30 was run. Vimentin was positive [Figure 5]. All other markers were negative. We rendered a diagnosis of pure intratesticular sarcoma and synchronous mediastinal sarcoma with lung metastases.
Figure 1: Gross photograph showing lungs with large, irregular, grey tan mediastinal fleshy mass encasing the left lung

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Figure 2: Testis replaced by a fleshy nodular tumor mass with extensive areas of necrosis (H and E, ×10)

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Figure 3: (a-d) Histopathological sections showing testicular tumor composed of spindle cells arranged in a fascicular pattern (H and E, ×10), hemangiopericytomatous pattern (H and E, ×10), myxoid areas with cells arranged in reticular pattern (H and E, ×10), and focal chondroid area (H and E, ×40)

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Figure 4: High power view showing pleomorphic bizarre tumor giant cells (H and E, ×40)

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Figure 5: Immunohistochemical study showed diffuse vimentin positivity

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  Discussion Top


Primary intratesticular sarcoma is a unique tumor with little information in the literature about its natural history and treatment protocol due to its rarity. [1] Mediastinal masses present earlier due to compressive symptoms. Sarcoma of the testis usually presents as a painless swelling. [5] Our index case, presented with symptoms of compression but had a simultaneous involvement of testis and medistinum. He did not give relative importance to the testicular mass as it was slow growing and painless. Testicular mass was evident only on physical examination. Morphologically, we found unclassified sarcoma of testis. In such cases, primary testicular sarcoma, germ cell tumor (GCT) with sarcomatous component (SC), and paratesticular rhabdomyosarcoma (RMS) are the tumors under differential diagnosis. [6],[7] Paratesticular tissue was free, hence, paratesticular sarcoma was ruled out. However, no GCT component was seen in multiple sections studied, hence, GCT with SC was ruled out. GCTs of ovary, mediastinum, testis, and intracranial cavity may develop as a SC, [4] although it is relatively common in mediastinal GCTs, it is rare, accounting for 3-6% in testicular GCTs. [1] IHC helped us confirm our diagnosis of pure intratesticular sarcoma by a negative desmin, myogenin, and SMA which ruled out smooth muscle origin, negative c-Kit ruled out seminoma, negative PLAP ruled out teratoma and negative CD30 ruled out embryonal carcinoma. The metastasis of sarcomatous component of testicular GCT to mediastinum is a well-known phenomenon. [4],[5] However, we could not establish whether testicular sarcoma was primary or secondary involvement as the deceased had neglected the testicular enlargement. Lung metastases appeared to be due to be contiguous spread from mediastinal tumor. RMS, spindle cell sarcoma, fibrosarcoma, osteosarcoma, leiomyosarcoma, chondrosarcoma, Kaposi sarcoma, and unclassified sarcoma of the testis have been reported. [1],[2],[3],[4],[5],[6],[7],[8] Very few pure testicular sarcomas have been reported in the literature. Sarcomas are derived from the supporting cells of testicular interstitium: Peritubular myoid cells, endothelial cells, smooth muscle cell, or primitive mesenchymal cell. There is lack of data on the natural history, histological criteria for diagnosis and treatment recommendations because of rarity of this disease. Most cases reported in the literature indicate that intratestiucular sarcoma may be an indolent tumor with a potential for cure if treated early. [5] Adjuvant chemotherapy with single-agent adriamycin or adriamycin plus ifosfamide has been the mainstay of treatment for advanced and metastatic soft tissue sarcomas from other sites. [8],[9] The same regimens can be used as palliative therapy for testicular and mediastinal sarcomas. On this premise, our patient was put on adriamycin, but he succumbed on the very next day. Rarity of the condition and hence, lack of literature makes it difficult to define and optimize therapy. [1],[2],[3],[4],[5],[6],[7],[8],[9]


  Conclusion Top


To conclude, pure intratesticular sarcomas are very rare and a thorough search for germ cell element is essential before diagnosing a pure intratesticular sarcoma. Concurrent medistinal sarcoma may appear with earlier manifestations due to compressive symptoms.


  Acknowledgement Top


Altaf A. Momin, Assistant Commissioner of Police, DG Office, Colaba, Mumbai. Financial and general support.

 
  References Top

1.Chandra A, Baruah RK, Ramanujam, Rajalakshmi KR, Sagar G, Vishwanathan P, et al. Primary intratesticular sarcoma. Indian J Med Sci 2001;55:421-8.  Back to cited text no. 1
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2.Ulbright TM, Loehrer PJ, Roth LM, Einhorn LH, Williams SD, Clark SA. The development of non-germ cell malignancies within germ cell tumors. A clinicopathologic study of 11 cases. Cancer 1984;54:1824-33.  Back to cited text no. 2
[PUBMED]    
3.Zukerberg LR, Young RH. Primary testicular sarcoma: A report of two cases. Hum Pathol 1990;21:932-5.  Back to cited text no. 3
    
4.Guo CC, Punar M, Contreras AL, Tu SM, Pisters L, Tamboli P, et al. Testicular germ cell tumors with sarcomatous components: An analysis of 33 cases. Am J Surg Pathol 2009;33:1173-8.  Back to cited text no. 4
    
5.Giridhar V, Kumar PB, Natarajan K, Hegde P. Testicular leiomyosarcoma with metastasis. Indian J Urol 2011;27:278-9.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Schoeneich G, Oehlmann U, Gustmann C, Albers P. Primary intratesticular sarcoma of the testis: A rare manifestation of testicular cancer. Scand J Urol Nephrol 2005;39:249-50.  Back to cited text no. 6
    
7.Lavallée LT, Roustan Delatour NL, Cagiannos I, Bélanger EC, Mai KT. A unique case of a sarcoma arising in a testicular non- seminomatous mixed germ cell tumour with a predominant yolk sac component. Can Urol Assoc J 2011;5:E81-3.  Back to cited text no. 7
    
8.Porcaro AB, Antoniolli SZ, Maffei N, Beltrami P, Bassetto MA, Curti P. Management of testicular seminoma advanced disease. Report on 14 cases and review of the literature. Arch Ital Urol Androl 2002;74:81-5.  Back to cited text no. 8
    
9.Peterson AC, Porter M, Porter J. Adult testicular sarcoma: Presentation, evaluation, and treatment. Urology 2002;59:294-5.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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