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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 1  |  Page : 88-90
Extra genital heterologous malignant mixed mullerian tumor of primary peritoneal origin


Department of Pathology, Krishna Institute of Medical Sciences, University, Karad, India

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   Abstract 

The case of an extragenital heterologous malignant mixed müllerian tumor (MMMT) of primary peritoneal origin occurring in a 76-year-old female is presented. A large tumor was seen between the uterus and rectosigmoid occupying the entire pelvis. The uterus, fallopian tubes and ovaries were uninvolved. The tumor was composed of carcinomatous areas showing endometrioid and serous papillary differentiation and sarcomatous areas showing cartilaginous differentiation. The extragenital primary MMMTs of the female peritoneum are thought to originate from the secondary müllerian system. This case is presented for its rarity. To the best our knowledge, this is the first case of extragenital MMMT of primary peritoneal origin in Indian literature.

Keywords: Extragenital tumors, malignant mixed müllerian tumors, carcinosarcomas, secondary müllerian system

How to cite this article:
Naniwadekar M R, Desai S R, Ranade R G, Kanetkar S R. Extra genital heterologous malignant mixed mullerian tumor of primary peritoneal origin. Indian J Pathol Microbiol 2009;52:88-90

How to cite this URL:
Naniwadekar M R, Desai S R, Ranade R G, Kanetkar S R. Extra genital heterologous malignant mixed mullerian tumor of primary peritoneal origin. Indian J Pathol Microbiol [serial online] 2009 [cited 2019 Dec 10];52:88-90. Available from: http://www.ijpmonline.org/text.asp?2009/52/1/88/44976



   Introduction Top


Malignant mixed müllerian tumors are neoplasms composed of carcinomatous and sarcomatous components and are being regarded as metaplastic, poorly differentiated adenocarcinoma. [1] They are rare tumors, most commonly arising in the endometrium, ovaries,  Fallopian tube More Detailss, cervix and vagina in decreasing order of frequency. [2] Extragenital primary peritoneal MMMTs are very rare and only 30 cases have been reported in literature. [3] They are highly aggressive neoplasms that occur in elderly postmenopausal women. [4] The tumors are thought to arise in the secondary müllerian system consisting of the abdominal and pelvic peritoneum and its underlying mesenchymal tissue.


   Case Report Top


A 76-year-old female presented with pain and a lump in the lower abdomen, decreased appetite and pain during defecation for the past 3 months. A vaginal examination revealed a fixed mass felt through the left fornix. A rectal examination revealed a mass anterior to the rectum (extra luminal mass) with mucosa free. A barium meal follow through showed small bowel loops displaced on one side due to a mass effect on the left side. A pelvic ultrasound revealed a large, lobulated mass in the pelvis with hyper echoic areas. A cytologic examination of the peritoneal fluid was positive for malignant cells.

An exploratory laparotomy was done; 500 ml of hemorrhagic fluid was found and a large cauliflower-like mass was seen between the uterus and rectosigmoid colon, adherent to the external wall of rectosigmoid and the back of the uterus and occupying the entire pelvis. The mass was resected with the uterus and adnexa and was sent for histopathologic study. The cytoreductive surgery was optimum.

Gross : The mass consisted of multiple grey-white, fleshy and irregular pieces of soft to firm tissue measuring in total 17 x 13 x 7 cm and weighing 400 gm [Figure 1]. The cut section showed a grey-white and grey-brown appearance with necrotic areas and gelations chondroid areas. The uterus, cervix and bilateral adnexae appeared unremarkable.

Microscopy : The tumor [Figure 2] was composed of a mixture of carcinomatous and sarcomatous components. The carcinoma component was formed of endometrioid and high-grade serous papillary carcinoma, along with diffuse sheets of undifferentiated cells. The stromal component was heterologous, with myxoid and chondromatous areas. Biopsies from the rectosigmoid wall showed tumor infiltration. Sections from the endomyometrium, fallopian tubes, cervix and ovaries were free from tumor. The immunohistochemical findings showed simultaneous coexpression of keratin and vimentin in both, the epithelial and mesenchymal components and the absence of desmin and estrogen and progesterone receptors. Postoperatively, the patient was given ifosfamide (1gm/m 2 x 5 days) and cisplatin (70 mg/m 2 ) chemotherapy. However, she died of septicemia and renal failure following marked neutropenia after the second course.


   Discussion Top


The occurrence of MMMT in an extragenital location is very rare. In the present case, the uterus, ovaries and fallopian tubes were unremarkable, thus the tumor was regarded as a primary extragenital MMMT arising from the pelvic peritoneum. The different sites for extragenital MMMTs are pelvic peritoneum, the serosal surface of the colon, retroperitoneum, antero-lateral abdominal peritoneum and omentum. [4] Ober and Black reported the first case of MMMT occurring in the rectovaginal peritoneum. [5] To the best of our knowledge, only 30 cases of extragenital MMMT have been described in English literature [3] and this is the first case in Indian literature.

Extragenital MMMTs arise from the secondary müllerian system as Lanchlan has stated, consisting of the abdominal and pelvic peritoneum and its underlying mesenchymal tissue, [2] which can give rise to any type of müllerian neoplasm. Tumors can arise from the malignant transformation of peritoneal endometriosis, endosalpingiosis, or endocervicosis and from the transformation of poorly differentiated epithelial neoplasms into mesenchymal differentiation. [2] The simultaneous coexpression of keratin and vimentin in carcinomatous and sarcomatous components gives support to conversion (metaplastic transformation) or the combination (origin from a common stem cell precursor) theories for histogenesis. The uterine MMMTs are being regarded as a metaplastic poorly differentiated adenocarcinoma; the sarcomatous component being a manifestation of increased aggressiveness and being derived from a carcinomatous element as a result of dedifferentiation. They are monoclonal neoplasms and not two separate malignant genotypes. [1]

Extragenital MMMT was found to be associated with synchronous or metachronous gynecologic tumors of müllerian duct origin, [6] previous irradiation, [7] and extragenital endometriosis. [8] In the present case, the patient had no history of an operation for any other tumors or previous irradiation. Microscopically, there was neither associated endometriosis nor did the tumor reveal positivity for estrogen or progesterone receptors.

Primary peritoneal MMMTs are highly aggressive tumors with a poor prognosis. [4] It has been proposed that the presence of high-grade serous or clear cell carcinomatous elements is associated with a higher frequency of metastases and poor outcome in uterine carcinosarcomas. [9],[1] But the similar observation is not documented for extragenital MMMTs. Garamvoelgyi, et al . have observed that there was no apparent relationship between the outcome and the presence or absence of a heterologous component in extragenital MMMT. [7]

The pathologic stage at the time of diagnosis has been shown to be the most reliable predictor of prognosis in uterine MMMT. Surgical excision is the most effective treatment in carcinosarcomas [5] and adjuvant radiotherapy and various combinations of chemotherapy yielded inconsistent results. [5] However, Wong, et al . have observed that patients with MMMTs of the uterus who underwent sequential adjuvant therapy using cisplatin and ifosfamide chemotherapy and radiotherapy had an improved survival rate. [10] The patient in this case had optimum cytoreductive surgery and was treated with postoperative chemotherapy of cisplatin and ifosfamide. However, she developed severe febrile neutropenia after the second course of chemotherapy and died of septicaemia.

This case is presented for its rarity. It demonstrates the pluripotentiality of female pelvic peritoneum to differentiate into tumors resembling those of the genital tract.

 
   References Top

1.McCluggage WG. Uterine carcinosarcomas (malignant mixed Müllerian tumors) are metaplastic carcinomas. Int J Gynecol Cancer 2002;12:687-90.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Mira JL, Fenoglio-Preiser CM, Husseinzadeh N. Malignant mixed müllerian tumor of the extraovarian secondary müllerian system: Report of two cases and review of the English literature. Arch Pathol Lab Med 1995;119;1044-9.  Back to cited text no. 2    
3.Ko ML, Jeng CJ, Huang SH, Shen J, Tzeng CR, Chen SC. Primary peritoneal carcinosarcoma (malignant mixed müllerian tumor): Report of a case with five year disease free survival after surgery and chemoradiation and a review of literature. Acta Oncol 2005;44:756-60.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Shen DH, Khoo US, Xue WC, Ngan HY, Wang JL, Liu VW, et al . Primary peritoneal malignant mixed müllerian tumors. A clinicopathologic, immunohistochemical and genetic study. Cancer 2001;91:1052-60.  Back to cited text no. 4    
5.Ober WB, Black MB. Neoplasms of the subcoelomic mesenchyme: Report of two cases. AMA Arch Pathol 1955;59:698-705.  Back to cited text no. 5  [PUBMED]  
6.Mikami M, Kuwabara Y, Tanaka K, Komiyama S, Ishikawa M, Hirose T. Malignant mixed müllerian tumor of primary mesenteric origin. Int J Gynecol Cancer 2005;15:1249-53.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Garamvoelgyi E, Guillou L, Gebhard S, Salmeron M, Seematter RJ, Hadji MH. Primary malignant mixed müllerian tumor (metalplastic carcinoma) of the female peritoneum: A clinical, pathologic and immunohistochemical study of three cases and a review of literature. Cancer 1994;74:854-63.   Back to cited text no. 7  [PUBMED]  
8.Noel JC, Anaf V, Fayt I, Wespes E. Ureteral müllerian carcinosarcoma (mixed müllerian tumor) associated with endometriosis occurring in a patient with a concentrated soy isoflavones supplementation. Arch Gynecol Obstet 2006;274:389-92.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Silverberg SG, Major FJ, Blessing JA, Fetter B, Askin FB, Liao SY, et al . Carcinosarcoma (malignant mixed mesodermal tumor) of the uterus: A gynecologic oncology group pathologic study of 203 cases. Int J Gynecol Pathol 1990;9:1-19.  Back to cited text no. 9  [PUBMED]  
10.Wong L, See HT, Khootan HS, Low JS, Ng WT, Low JJ. Combined adjuvant cisplatin and ifosfamide chemotherapy and radiotherapy for malignant mixed müllerian tumors of the uterus. Int J Gynecol Cancer 2006;16:1364-9.   Back to cited text no. 10    

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Correspondence Address:
M R Naniwadekar
Naniwadekar Hospital, Market Yard, Shaniwar Peth, Karad 415 110, Satara, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.44976

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    Figures

  [Figure 1], [Figure 2]

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