|Year : 2016 | Volume
| Issue : 3 | Page : 367-369
Large retroperitoneal malignant mixed Mullerian tumor
Shailaja Prabhala1, Jayashankar Erukkambattu1, Haripriya Vedantham2, Ramamurti Tanikella1
1 Department of Pathology, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India
2 Department of Obstetrics and Gynecology, Kamineni Academy of Medical Sciences and Research Centre, Hyderabad, Telangana, India
|Date of Web Publication||17-May-2016|
H. No. 8-14/1, Ravindranagar Colony, Street No. 8, Habsiguda, Hyderabad - 500 007, Telangana
Source of Support: None, Conflict of Interest: None
A 57-year-old asymptomatic lady had come for a master health check-up with a report of fibroid uterus diagnosed on ultrasound at a private hospital. Intraoperatively, a large retroperitoneal tumor was found with the uterus and adnexae being normal. The mass was removed in toto, and its pathological examination revealed a malignant mixed Mullerian tumor (MMMT). Immunohistochemistry also supported the diagnosis of MMMT. The postoperative period was uneventful, and the patient was discharged with the advice of oncology referral.
Keywords: Biphasic tumors, immunohistochemistry, malignant mixed Mullerian tumor, retroperitoneal
|How to cite this article:|
Prabhala S, Erukkambattu J, Vedantham H, Tanikella R. Large retroperitoneal malignant mixed Mullerian tumor. Med J DY Patil Univ 2016;9:367-9
|How to cite this URL:|
Prabhala S, Erukkambattu J, Vedantham H, Tanikella R. Large retroperitoneal malignant mixed Mullerian tumor. Med J DY Patil Univ [serial online] 2016 [cited 2022 Aug 11];9:367-9. Available from: https://www.mjdrdypu.org/text.asp?2016/9/3/367/182509
| Introduction|| |
Malignant mixed Mullerian tumors (MMMT) are rare malignancies of the female genital tract contributing <1% for all gynecologic malignancies. MMMTs of extragenital origin are still rarer.  They are highly aggressive tumors which usually present with abdominal symptoms and affect postmenopausal women. , Our case had a completely asymptomatic presentation in spite of a large retroperitoneal mass and had no involvement of other organs. We present this case due to the rarity of the tumor and its unusual presentation.
| Case Report|| |
A 57-year-lady had come for a routine health check-up. She did not have any complaints. She had a history of increased frequency and burning micturition 1-month ago for which an ultrasound examination was done at a private clinic which showed a large subserosal uterine fibroid. She was para 2 and live births 2, both full term, normal vaginal deliveries. The last childbirth was 31 years ago followed by bilateral tubal ligation. She had attained menopause 12 years ago. She was diabetic and hypertensive for 10 years and was on regular oral medication. She had come to our hospital for routine investigations and to address the issue of fibroid uterus.
On examination, the patient had an average built. Her general and systemic examinations were within normal limits except for per abdomen examination which showed a uterus of 14-16 weeks size and nontender. There was no ascites or pleural effusion. Per speculum/vaginum examination showed healthy cervix and vagina. The uterus was uniformly enlarged and the fornices were free and mobile. Ultrasound examination done in our institute showed a large mass measuring 14.5 cm × 5.5 cm toward the right lateral aspect of the uterus, possibly in the pouch of Douglas which felt as a subserosal fibroid [Figure 1]a. The uterus measured 7.5 cm × 5 cm × 3 cm. The right ovary was not visualized, and the left ovary was of normal size. Computerized tomography was advised but could not be done.
|Figure 1: (a) The ultrasound scan shows a large mass abutting posterior wall of the uterus. (b) The tumor is well-circumscribed, solid, and has a hemorrhagic fleshy cut surface. (c) Areas of ciliated glandular cells with eosinophilic cytoplasm (H and E, ×400). (d) Glandular cells exhibiting pleomorphism and mitoses (H and E, ×400)|
Click here to view
On investigation, her preoperative workup was within normal limits. The cervical smear was normal. She was taken for abdominal hysterectomy under spinal anesthesia. Intraoperatively, a large mass was seen arising from pelvic retroperitoneum measuring 12 cm × 10 cm. Flimsy adhesions were present between the mass and sigmoid colon. The uterus and both ovaries were appeared normal. The mass was removed in toto along with infracolic omentectomy and both the ovaries were biopsied. The liver, spleen, intestines, and bilateral pelvic lymph nodes were explored for any nodules, but they appeared normal.
On pathology, a large, smooth, well-circumscribed mass measuring 12 cm × 10 cm × 10 cm was received. Its cut surface had solid, variegated appearance with areas of hemorrhage, and necrosis [Figure 1]b. The ovarian tissue bits were grayish, unremarkable, and were all embedded. The omental tissue appeared normal.
The histopathology of the mass revealed a tumor having biphasic appearance [Figure 1]c, d, and [Figure 2]a. The epithelial component was made up of glands, trabeculae, microcysts with secretions, small clusters of ciliated and nonciliated round to polygonal cells with distinct cell borders, eosinophilic granular cytoplasm, and pleomorphic vesicular nuclei. The spindle cells were short, plump, and bland to pleomorphic with occasional mitotic figures. Spotty calcification, fibrosis, and foci of necrosis were present. No heterologous elements were seen. Light microscopy was suggestive of MMMT, homologous type. Both the ovaries and the omentum showed normal morphology. Immunohistochemistry (IHC) [Table 1] and [Figure 2]b-d confirmed the diagnosis of MMMT. The postoperative period was uneventful, and she was discharged on the 5 th postoperative day, and was advised oncologic referral but was lost to follow-up.
|Figure 2: (a) Short spindled stromal cells with mild pleomorphism (H and E, ×400). (b) Pancytokeratin positivity in epithelial cells and in focal spindle cells (H and E, ×400). (c) Strong vimentin positivity in epithelial and spindle cells (H and E, ×100). (d) Calretinin positivity in epithelial cells (H and E, ×100)|
Click here to view
| Discussion|| |
MMMT outside the female genital tract was described for the first time by Ober and Black in 1955.  As a primary tumor in the retroperitoneum, they are extremely rare but commonly occur in postmenopausal women, and present with symptoms of abdominal distension, pain, and palpable mass.  In contrast, our case was completely asymptomatic, and the mass was incidentally detected. Huang et al. have reviewed the age for MMMT and found the median age to be 62.8 years (range: 33-87 years).  Our case had none of the risk factors associated with MMMT such as history of pelvic irradiation, obesity, nulliparity, estrogen exposure, endometriosis, and synchronous or metachronous gynecologic or nongynecologic malignancies. , In our case, the uterus, cervix, ovaries, and the surrounding structures were thoroughly explored and appeared absolutely normal as expected for retroperitoneal MMMT.  Spinelli et al.  reviewed seven cases of retroperitoneal MMMTs which were solid to cystic and whose size ranged from 9 to 26 cm in the largest dimension. Our case also had a similar size of 12 cm × 10 cm × 10 cm and was completely solid.
The proposed theories which explain the biphasic appearance of the tumor are the "collision," "combination," and the most widely accepted "conversion" theory  and also origin from the "secondary Mullerian system" or from residual hormone-independent cells of Mullerian ducts in the retroperitoneum. , The MMMTs are biphasic tumors having malignant epithelial and mesenchymal components. On IHC,  the epithelial component is positive for cytokeratin 7, negative for cytokeratin 20, and may show vimentin positivity. The spindle cells are vimentin positive and may also show cytokeratin 7 positivity. Our IHC results were as expected in MMMT. An unusual observation was that our case showed good positivity for calretinin. Various benign and malignant entities can enter the differential diagnosis like large hydatid cysts, especially when they show calcifications on imaging, subserosal leiomyomas, retroperitoneal soft tissue sarcomas, malignancies of the kidney, adrenal glands, and germ cell tumors, etc. Mesothelioma is a close differential diagnosis, but considering the asymptomatic presentation, postmenopausal status, absence of ascites, the large size of the tumor, its circumscribed and solid nature, and most importantly the presence of ciliated cells in glandular pattern, the diagnosis of MMMT was entertained.
| Conclusion|| |
Primary retroperitoneal MMMTs are common in postmenopausal age. They can grow to a large size without causing any symptoms. Though rare, they have to be considered in the differential diagnosis of asymptomatic retroperitoneal tumors, especially in female patients.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Huang CC, Ma CJ, Huang WT, Chan TF, Wang JY. Primary malignant mixed Müllerian tumor arising from the mesorectum with a synchronous ovarian cancer: A case report and review of the literature. J Med Case Rep 2011;5:15.
de León DC, Pérez-Montiel D, Chanona-Vilchis J, Dueñas-González A, Villavicencio-Valencia V, Zavala-Casas G. Primary retroperitoneal mucinous cystadenocarcinoma: Report of two cases. World J Surg Oncol 2007;5:5.
Ober WB, Black MB. Neoplasms of the subcoelomic mesenchyme; report of two cases. AMA Arch Pathol 1955; 59:698-705.
Kuyumcuoglu U, Kale A. Homologous type of malignant mixed Mullerian tumor of the uterus presenting as a cervical mass. J Chin Med Assoc 2009;72:533-5.
Shintaku M, Matsumoto T. Primary Mullerian carcinosarcoma of the retroperitoneum: Report of a case. Int J Gynecol Pathol 2001;20:191-5.
Spinelli C, Strambi S, Tartaglia D, Di Franco G, Pucci V, Faviana P, et al.
Primary retroperitoneal müllerian adenocarcinoma: A case report and literature review. Case Rep Oncol 2013;6:616-21.
McCluggage WG. Malignant biphasic uterine tumours: Carcinosarcomas or metaplastic carcinomas? J Clin Pathol 2002;55:321-5.
Elnemr A, Yonemura Y, Shinbo M, Nishino E. Primary retroperitoneal Mullerian adenocarcinoma. Rare Tumors 2010;2:e6.
Kurshumliu F, Rung-Hansen H, Skovlund VR, Gashi-Luci L, Horn T. Primary malignant mixed Müllerian tumor of the peritoneum a case report with review of the literature. World J Surg Oncol 2011;9:17.
[Figure 1], [Figure 2]