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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 262-264
Extraskeletal mesenchymal chondrosarcoma at unusual location involving spleen and kidney with review of literature

Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

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Date of Web Publication19-Jun-2017


Mesenchymal chondrosarcoma (MC) is a rare malignant neoplasm bearing characteristic dimorphic pattern histologically. We describe two rare cases of primary MC involving two different visceral organs (1) a 24-year-old man with solid renal mass and, (2) a 42-year-old man with cystic splenic mass. The histological and immunophenotypical features of both lesions were classical of MC. Although this lesion is uncommon in visceral organs, the possibility of this rare entity must be kept in differential diagnosis with compatible morphology.

Keywords: Kidney, mesenchymal chondrosarcoma, spleen

How to cite this article:
Pani KC, Yadav M, Priyaa P V, Kumari N. Extraskeletal mesenchymal chondrosarcoma at unusual location involving spleen and kidney with review of literature. Indian J Pathol Microbiol 2017;60:262-4

How to cite this URL:
Pani KC, Yadav M, Priyaa P V, Kumari N. Extraskeletal mesenchymal chondrosarcoma at unusual location involving spleen and kidney with review of literature. Indian J Pathol Microbiol [serial online] 2017 [cited 2020 Feb 24];60:262-4. Available from: http://www.ijpmonline.org/text.asp?2017/60/2/262/208380

   Introduction Top

Mesenchymal chondrosarcoma (MC) was first described by Lichtenstein and Bernstein [1] in 1959, which is a rare cartilaginous neoplasm predominately occurring in extraskeletal sites commonly in the head and neck. Histologically, MC has a characteristic dimorphic pattern, composed of islands of hyaline cartilage admixed with highly undifferentiated small round cells.[2],[3] The biological behavior of MC is generally aggressive. The treatment protocol of this tumor has not been well established because of its rarity.[4] Visceral organs are rare sites of occurrence of this neoplasm. Only sporadic cases of MC involving the visceral organs have been described. We present two extremely rare locations of the primary MC involving two different visceral organs.

   Case Reports Top

Case report 1

A 24-year-old male presented with intermittent nonradiating right flank pain for 2 months. He had history of right inguinal hernia repair 3 years back. There was no history of hematuria, dysuria, lithuria, or lower urinary tract symptoms. Ultrasound abdomen showed a large, well-defined heterogeneous hypoechoic mass measuring 8.5 cm × 6.8 cm involving mid and upper pole of the right kidney. Foci of calcification and vascularity were noted. Contrast-enhanced computed tomography (CECT) of abdomen revealed a large mass at the mid and upper pole of the right kidney with foci of calcification and cystic spaces. The patient underwent right laparoscopic radical nephrectomy.

Gross specimen of the right kidney showed a large lobulated growth measuring 8 cm × 7 cm × 6 cm involving middle and upper pole of the kidney. Cut surface of the growth was gray-brown in appearance with areas of calcifications [Figure 1]a. Light microscopy revealed a lobulated tumor in the subcapsular region composed of sheets of small round cells having round to irregular nuclei, fine chromatin, and scant cytoplasm with interspersed thin-walled ectatic blood vessels in a hemangiopericytomatous pattern [Figure 2]a,[Figure 2]b,[Figure 2]c. Large islands of mature cartilage with foci of calcification were seen. The tumor was limited to renal parenchyma. Renal sinus, vessels, and ureter were free of tumor. The patient was put on four cycles of combined chemotherapy (ifosfamide + doxorubicin). At a follow-up period of 6 months, the patient was symptom-free.
Figure 1: (a) Cut surface of nephrectomy specimen showing well-defined tumor composed of cartilaginous and gray-brown areas. (b) Cut surface of splenectomy specimen a tumor with multiple cystic spaces. Part of normal splenic parenchyma is seen in the left lower region

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Figure 2: (a) Low-power microphotograph showing hemangiopericytomatous pattern of blood vessels (H and E, ×10). (b) Islands of mature cartilage surrounded by small round tumor cells (H and E, ×20). (c) High-power photomicrograph showing tumor cells with round to irregular nuclei, fine chromatin, and scant cytoplasm (H and E, ×40)

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Case report 2

A 42-year-old male presented with progressively increasing pain in the left hypochondrium for 6 months. Physical examination revealed large palpable lump in the left upper quadrant of abdomen as well as the right inguinoscrotal swelling reaching up to apex of scrotum. CECT abdomen showed a heterogeneously enhancing multiseptate solid cystic space occupying lesion measuring 18 cm × 15 cm × 13 cm in the spleen with necrotic areas and calcifications. The lesion was displacing the stomach and pancreatic tail anteriorly and the left kidney inferiorly. The patient underwent splenectomy with distal pancreatectomy and meshplasty of the right inguinal hernia.

Grossly spleen measured 22 cm × 12 cm × 7 cm and weighed 2400 g. The outer surface was bosselated. The cut surface showed a multiloculated solid cystic lesion measuring 16 cm × 14 cm × 7 cm occupying the hilum of the spleen [Figure 1]b. The cysts were filled with serous fluid. Solid gray-white areas along with areas of hemorrhage and necrosis were noted.

Histology showed a partially encapsulated neoplasm arranged in sheets with interspersed thin-walled ectatic vascular spaces at places. The tumor cells displayed round to oval nuclei with fine chromatin, conspicuous nucleoli, focal nuclear grooving, and moderate amount of cytoplasm. Few mitotic figures and foci of chondroid differentiation were also noted. Cystic spaces were filled with luminal secretions and lined by flattened cells.

On immunohistochemistry (IHC), tumor cells were uniformly positive for vimentin and CD-99, and cartilaginous islands were positive for S-100 [Figure 3]a,[Figure 3]b,[Figure 3]c. CD34 expression was seen only in the vessel wall. Desmin and bcl-2 were negative. This patient was lost to follow-up.
Figure 3: Immunohistochemistry for vimentin (a) and CD-99 (c) diffusely positive in tumor cells. S-100 (b) is positive in cartilaginous island (IHC, ×40)

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   Discussion Top

MC is a rare malignant tumor, arising largely from extraskeletal sites, although it involves skeletal tissues in minority of cases. Approximately, one-fifth to one-third of the lesions arises from somatic soft tissues. Although head and neck is the most common site, extraskeletal MC has been reported from thoracic, abdominal, and retroperitoneal organs.[2] MC can occur both in pediatric and adult population.[2],[3],[4] Since the first case report by Malhotra et al., only a handful of cases of primary MC of the kidney have been described.[5],[6] Only single case of primary MC of human spleen has been reported by Rossetto et al.[7]

The gross features of MC include solid cystic soft to firm usually well-circumscribed lesions ranging from 3 to 30 cm in diameter. Prominent cartilaginous appearance, as well as foci of necrosis and hemorrhage, may be prominent. Histologically, MC is characterized by a biphasic histologic pattern that is composed of small undifferentiated round cells admixed with islands of well-differentiated hyaline cartilage. Hemangiopericytoma-like pattern is a common finding.[2],[3],[4] On IHC, the undifferentiated cells are usually positive for vimentin and CD99, whereas cells in chondroid areas are positive for S100.[2] Sox9, a master regulator for cartilage development, is considered as the single most important IHC marker for distinguishing MC from other small round cell tumors. Sox9 expression has been observed in both cartilaginous and mesenchymal regions.[8] The diagnosis of MC may be aided by its positivity for D2-40 (podoplanin).[9] The main use of D2-40 in diagnostic pathology is as a marker of lymphatic endothelium; hence aids in diagnosing additional foci of lymphovascular invasion. A Robertsonian translocation involving chromosomes 13 and 21 has been detected in two cases of MC.[10] Compatible histological and IHC features were observed in both of our cases.

Extraaxial MC is considered as an aggressive neoplasm and is associated with poor prognosis. Local recurrence and metastases are common. Surgical resection with adequate margins is considered as gold standard of treatment.[4] The role of chemotherapy and radiotherapy is controversial. The previous reports suggest that MC is relatively sensitive to doxorubicin-based combination chemotherapeutic regimens and case 1 in the present series received similar chemotherapy who was symptom-free after 6 months of follow-up. MCs in pediatric population tend to be more extraosseous and are associated with better outcome.[11]

   Conclusion Top

We report two highly unusual cases of MC in kidney and spleen. Although uncommon, the possibility of this rare entity must be kept in solid cystic lesions of visceral organs. Due to dismal prognosis and a higher chance of recurrence as well as distant metastasis, these patients should be properly followed up.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Lightenstein L, Bernstein D. Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 1959;12:1142-57.  Back to cited text no. 1
Nakashima Y, Park YK, Sugano O. Mesenchymal chondrosarcoma. In: Fletcher CD, Unni KK, Mertens F, editors. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon, France: IARC Press; 2002. p. 255-6.  Back to cited text no. 2
Nakashima Y, Unni KK, Shives TC, Swee RG, Dahlin DC. Mesenchymal chondrosarcoma of bone and soft tissue. A review of 111 cases. Cancer 1986;57:2444-53.  Back to cited text no. 3
Xu J, Li D, Xie L, Tang S, Guo W. Mesenchymal chondrosarcoma of bone and soft tissue: A systematic review of 107 patients in the past 20 years. PLoS One 2015;10:e0122216.  Back to cited text no. 4
Malhotra CM, Doolittle CH, Rodil JV, Vezeridis MP. Mesenchymal chondrosarcoma of the kidney. Cancer 1984;54:2495-9.  Back to cited text no. 5
Tyagi R, Kakkar N, Vasishta RK, Aggarwal MM. Mesenchymal chondrosarcoma of kidney. Indian J Urol 2014;30:225-7.  Back to cited text no. 6
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Rossetto A, Saccomano E, Zompicchiatti A, Avellini C, Toffoli S, Miolo G, et al. Mesenchymal chondrosarcoma of the spleen: Report of a case. Tumori 2011;97:e10-5.  Back to cited text no. 7
Fanburg-Smith JC, Auerbach A, Marwaha JS, Wang Z, Rushing EJ. Reappraisal of mesenchymal chondrosarcoma: Novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases. Hum Pathol 2010;41:653-62.  Back to cited text no. 8
Daugaard S, Christensen LH, Høgdall E. Markers aiding the diagnosis of chondroid tumors: An immunohistochemical study including osteonectin, bcl-2, cox-2, actin, calponin, D2-40 (podoplanin), mdm-2, CD117 (c-kit), and YKL-40. APMIS 2009;117:518-25.  Back to cited text no. 9
Naumann S, Krallman PA, Unni KK, Fidler ME, Neff JR, Bridge JA. Translocation der (13;21)(q10;q10) in skeletal and extraskeletal mesenchymal chondrosarcoma. Mod Pathol 2002;15:572-6.  Back to cited text no. 10
Dantonello TM, Int-Veen C, Leuschner I, Schuck A, Furtwaengler R, Claviez A, et al. Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults: Experiences of the CWS and COSS study groups. Cancer 2008;112:2424-31.  Back to cited text no. 11

Correspondence Address:
Niraj Kumari
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 266 014, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_16_16

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