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CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 2  |  Page : 172-174

Primary carcinosarcoma of the spleen – A rare tumor


Department of Pathology, Government Medical College, Kottayam, Kerala, India

Date of Submission18-Dec-2019
Date of Decision24-Feb-2020
Date of Acceptance01-May-2020
Date of Web Publication21-Jul-2020

Correspondence Address:
Jayalakshmy Payippat Leelamma
Department of Pathology, C Block, Government Medical College, Kottayam - 686 008, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sjhs.sjhs_68_19

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  Abstract 


A 65-year-old woman with an acute abdomen underwent emergency laparotomy and excision of the spleen. Pathologic examination revealed a neoplasm in the spleen composed of an intimate admixture of malignant epithelial and stromal elements. In the absence of a neoplasm elsewhere, the final diagnosis rendered was primary splenic carcinosarcoma. This article describes the gross and histopathological features of this rare splenic tumor in a 65-year-old female and discusses the possible origin and pathogenesis. The surgery is the mainstay of treatment and prognosis is dismal. Given the rarity of this tumor in the spleen, reporting this case will add to the scarce data currently available.

Keywords: Carcinosarcoma, primary, prognosis, rare, splenic


How to cite this article:
Leelamma JP, Sujatha D, Davis NT. Primary carcinosarcoma of the spleen – A rare tumor. Saudi J Health Sci 2020;9:172-4

How to cite this URL:
Leelamma JP, Sujatha D, Davis NT. Primary carcinosarcoma of the spleen – A rare tumor. Saudi J Health Sci [serial online] 2020 [cited 2020 Sep 29];9:172-4. Available from: http://www.saudijhealthsci.org/text.asp?2020/9/2/172/290321




  Introduction Top


There are two broad categories of neoplasms in the spleen: lymphoid and nonlymphoid. Lymphoid neoplasms are common, whereas primary and secondary nonlymphoid neoplasms are rare.[1] The most common nonlymphoid neoplasms are of vascular origin which include hemangioma, littoral cell angioma, lymphangioma, hemangioendothelioma, and angiosarcoma. The most common nonlymphoid malignant neoplasm occurring in the spleen is angiosarcoma. The involvement of the spleen by metastasis is uncommon and is thought to be due to a lack of afferent lymphatics.[2] Although primary splenic carcinomas have been documented in literature since 1951, none of them showed a concomitant malignant stromal (sarcomatous) component.[3]


  Case Report Top


A 65-year-old female was admitted to the General Surgery department of the Government Medical College Kottayam for the investigation of the left upper abdominal pain and abdominal distension of 7 months duration. There was a history of weight loss and loss of appetite. Examination revealed splenomegaly 3 cm below the left costal margin. There was no evidence of hepatomegaly or free fluid in the abdomen. Systemic examination was unremarkable. There was no generalized lymphadenopathy. Blood investigations revealed microcytic hypochromic anemia with normal leukocyte and platelet counts. Viral markers were negative. Renal and liver function tests were normal.

Abdominal ultrasound showed an enlarged spleen with a mass of abnormally high echogenicity but no ascites. Contrast-enhanced computed tomography (CT) of the abdomen showed a high-density mass in the spleen measuring 11 cm × 8 cm × 10 cm almost involving entire spleen accompanied by multiple cystic areas. The liver showed multiple peripherally enhancing hypodense lesions in both lobes largest measuring 3 cm × 3 cm × 3 cm. There was no para-aortic or pelvic lymphadenopathy. The gall bladder, bilateral kidneys, adrenals, ovaries, and urinary bladder were within the normal limits. A diagnosis of primary splenic malignancy with liver metastasis was considered.

An exploratory laparotomy was performed which showed an enlarged spleen and multiple yellowish lesions on the surface of the liver. The rest of the internal organs were normal. Splenectomy was performed, and incision biopsy was taken from lesions in the liver. The resected spleen weighed 300 g and measured 14 cm × 12 cm × 10 cm. The outer surface was yellowish-brown. On sectioning the parenchyma showed an ill-circumscribed gray-white growth almost replacing spleen measuring 10 cm × 6 cm × 8 cm with areas of necrosis, hemorrhage, and cystic degeneration [Figure 1]a.
Figure 1: (a) Gross specimen of the spleen showing tumor involving the entire organ. (b) Malignant gland surrounded by malignant mesenchymal component (H and E, ×200). (c) Malignant glandular component (H and E, ×200). (d) Combination of the malignant gland and stroma (H and E, ×200)

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Histopathology of the growth showed a malignant neoplasm composed of intimately admixed epithelial and mesenchymal components [Figure 1]b. Epithelial component showed cells in glandular [Figure 1]b and papillary patterns [Figure 1]c. Individual cells are columnar and pleomorphic with a moderate amount of pale eosinophilic cytoplasm and pleomorphic nucleus with coarse chromatin and prominent nucleolus. Mucin was noted in the glandular lumen. Mesenchymal component showed spindle cells in whorls and fascicles [Figure 1]d. Cells have moderate eosinophilic cytoplasm and pleomorphic, spindle-shaped, and hyperchromatic nucleus. Osteoclast-like giant cells were seen interspersed between the neoplastic cells. The neoplasm showed extensive necrosis. The epithelial component was positive for cytokeratin [Figure 2]a and the mesenchymal component was positive for vimentin [Figure 2]b by immunohistochemical study. Histopathology of the lesion in the liver showed dense neutrophilic infiltrate suggestive of an abscess with no evidence of neoplasm.
Figure 2: (a) Epithelial component is positive for cytokeratin (IHC, ×200). (b) Sarcomatous area is positive for vimentin (IHC, ×200)

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With the above features, it was diagnosed as carcinosarcoma. As ancillary tests failed to show a primary site, a final diagnosis of primary splenic carcinosarcoma was performed. The patient succumbed to her disease a month later.


  Discussion Top


Carcinosarcomas are malignant tumors composed of a mixture of high-grade carcinoma and sarcoma. These tumors can arise in the different organs such as skin, salivary glands, lungs, esophagus, stomach, colon, uterus, ovaries, female peritoneum, head and neck, urinary tract, prostate, thyroid, and breast.[4]

Primary and secondary nonlymphoid tumors are rare in the spleen and even rarer is the occurrence of primary carcinosarcoma. Preoperative and postoperative examinations, such as detailed physical examination, abdominal ultrasound, CT scan of the thorax, abdomen, pelvis, and endoscopic examination of the gastrointestinal tract have to be conducted to exclude a metastasis before arriving at a diagnosis of primary carcinosarcoma.[3]

It has been postulated that the mesothelium of the spleen is the potential site for primary carcinosarcoma, because of the ability of the female peritoneum to undergo metaplasia and neoplasia.[5],[6] For the most extragenital carcinosarcomas, the neoplasm is believed to arise directly from the multipotential mesothelium.[6]

There are two important theories that explain the histological features of this tumor, namely the multiclonal and monoclonal theories.[4] The multiclonal theory states that two separate and independent clones of cells are involved which proliferate and merge as a single tumor, whereas the monoclonal theory postulates that these tumors develop as a result of the ability of the undifferentiated, totipotent neoplastic cells to undergo multiple pathways of terminal differentiation into histologically recognizable epithelial and mesenchymal elements.[4],[6] This theory is supported by the presence of immunoreactivity for epithelial markers (CK or EMA) in mesenchymal areas, presence of ultrastructural features (desmosomes or tonofilaments) of epithelial differentiation in sarcomatoid elements and genetic studies evaluating clonality.

The epithelial component in carcinosarcoma is in the form of adenocarcinoma or a squamous cell carcinoma. The homologous sarcomatous component is fibrosarcoma, and the heterologous component is chondrosarcoma, rhabdomyosarcoma, or osteosarcoma. The present case had adenocarcinoma elements with homologous sarcomatous differentiation and plenty of osteoclast-like giant cells. Only five cases have been reported in literature till 2017.[2],[3],[4],[5],[6] The treatment of choice for carcinosarcoma is surgical excision and adjuvant radiotherapy and various combinations of chemotherapy have yielded inconsistent results.[7]

In conclusion, splenic carcinosarcoma is a rare tumor which supposedly takes origin from the surface mesothelium of the spleen. Before rendering such a diagnosis, it is crucial to exclude metastasis from other primary sites because of its rarity. There are no specific treatment guidelines. The surgery is the mainstay of treatment and it has a dismal prognosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

The authors thank the paramedical staff Miss Kalakumari and Miss Suja who provided with the technical help.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Fotiadis C, Georgopoulos I, Stoidis C, Patapis P. Primary tumors of the spleen. Int J Biomed Sci 2009;5:85-91.  Back to cited text no. 1
    
2.
Kaza RK, Azar S, Al-Hawary MM, Francis IR. Primary and secondary neoplasms of the spleen. Cancer Imaging 2010;10:173-82.  Back to cited text no. 2
    
3.
Sun T, Wang GF, Zhang Y. Primary splenic carcinosarcoma with local invasion of chest wall: A rare case. J Zhejiang Univ Sci B 2017;18:717-22.  Back to cited text no. 3
    
4.
Kochar K, Vijayasekar C, Pandey U, Bhogal R, Brown L, Mathew G. Primary carcinosarcoma of the spleen: Case report of a rare tumor and review of the literature. Int J Surg Pathol 2009;17:72-7.  Back to cited text no. 4
    
5.
Rao A, Mathew G, Marimuthu K, Pandey U, Worlding J. Carcinosarcoma of the spleen: Literature review of a very rare tumour. Cent Eur J Med 2007;2:230-5.  Back to cited text no. 5
    
6.
Dave KS, Chauhan A, Bhansali R, Arora R, Purohit S. Uterine carcinosarcomas: 8-year single center experience of 25 cases. Indian J Med Paediatr Oncol 2011;32:149-53.  Back to cited text no. 6
  [Full text]  
7.
Yan JF, Kuang TT, Ji DY, Xu XW, Wang DS, Zhang RC, et al. Laparoscopic versus open distal pancreatectomy for benign or premalignant pancreatic neoplasms: A two-center comparative study. J Zhejiang Univ Sci B 2015;16:573-9.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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