Cancer Therapy Vol 2, 571-574, 2004

 

Primary peritoneal malignant mixed mullerian tumor (MMMT): a case report

Case Report

 

Marcelo Carraro Nascimento1, Poh See Choo2, Judy Bligh3, Andreas Obermair1,*

1Queensland Centre for Gynaecological Cancer, Royal Brisbane and WomenÕs Hospital, 6th Floor, Ned Hanlon Building, Butterfield Street, Herston QLD 4029, Australia

2Greenslopes Specialist Centre, Greenslopes Hospital, Newdegate Street, Greenslopes QLD 4120, Australia

3Sullivan Nicolaides Pathology, 134 Whitmore Street, Taringa, QLD 4068, Australia

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*Correspondence: A/Prof. Andreas Obermair, Queensland Centre for Gynaecological Cancer, Royal Brisbane and WomenÕs Hospital, 6th Floor, Ned Hanlon Building, Butterfield Street, Herston QLD 4029, Australia; Phone ++61 7 3636 8501; Fax ++61 7 3636 5289; E-mail: andreas_obermair@health.qld.gov.au

Key words: Primary peritoneal malignant mixed mullerian tumor, Histopathologic findings

Abbreviations: malignant mixed mullerian tumor (MMMT);

 

Received: 2 December 2004; Accepted: 28 January 2005; electronically published: March 2005

 

Summary

Primary Peritoneal Malignant Mixed Mullerian Tumours (MMMT) are extremely rare and our knowledge about the effectiveness of surgery and postoperative treatment is limited. A case of Primary Peritoneal MMMT in an 86-year old patient is reported. She presented with ascites, abdominal mass and elevated CA125 serum level. Due to medical impairment she received neoadjuvant single agent carboplatin, which was ineffective. After adding paclitaxel she had a remarkable response with improvement of symptoms and decline of CA125 levels. Subsequent optimal debulking revealed primary peritoneal MMMT. We believe that the addition of paclitaxel to carboplatin was crucial to both, improve the symptoms and minimise the disease preoperatively. This case should encourage investigators to evaluate the role of paclitaxel as part of combination chemotherapy of MMMT.

 

I. Introduction

First described in 1955, Primary Peritoneal Malignant Mixed Mullerian Tumours (MMMT) are extremely rare (Ober and Black, 1955). To date, approximately 30 cases with MMMT arising from the pelvic peritoneum, the pelvic wall, or omentum have been reported (Shen et al, 2001). Little is known about its histogenesis and pathogenesis. A possible association between endometriosis and primary peritoneal MMMT was described (Rose, 1997). There is no standard treatment and our knowledge about the effectiveness of surgery and postoperative treatment is limited. The prognosis is extremely poor and most patients die of disease within a year from diagnosis (Garamvoelgyi, 1994). We describe a case of primary peritoneal MMMT treated with neoadjuvant chemotherapy followed by surgery.

 

II. Case report

Mrs W.B., 86 years of age, presented in October 2003 with increasing abdominal distension and discomfort. CT and ultrasound of the pelvis/abdomen demonstrated a large intra-abdominal mass, disseminated disease and ascites. Her Chest X-Ray was clear. Her CA125 was 720 U/mL and CEA was 1.3 mcg/L at presentation.

Her surgical history included removal of a squamous cell carcinoma of her nose and a total hip replacement. Medically she was troubled by ischaemic heart disease, hypertension, high cholesterol levels, and osteoporosis. She had no history of lung disease.

On general examination Mrs. W.B. was short of breath on very minor levels of exertion. Her abdomen was distended due to ascites. A small umbilical hernia was noted and on internal examination a mass could be felt in the pelvis, and nodularity was noted in the pouch of Douglas. A paracentesis revealed cells highly suspicious of adenocarcinoma. The diagnosis of advanced ovarian cancer was assumed.

The patient was felt not to be fit for major pelvic surgery. Neo-adjuvant chemotherapy with single-agent carboplatin (360 mg/m2) was administered for two cycles. During this treatment her CA 125 increased to 1311 U/mL. Chemotherapy was changed to carboplatin (360 mg/m2) plus paclitaxel (175 mg/m2) for another 5 cycles. During this combination chemotherapy her general health improved dramatically and her CA125 levels dropped to 67 U/mL in January 2004. Surgical debulking in February 2004 revealed an umbilical metastasis of 2 cm and several pelvic masses of up to 11 cm, which were firmly attached to the rectosigmoid colon. There was miliary disease on the peritoneal surfaces of the pelvic peritoneum but the peritoneum of the upper abdomen was clear. The uterus and the ovaries looked macroscopically uninvolved. A total hysterectomy, bilateral salpingo-oophorectomy, pelvic and aortic lymph node dissection, resection of pelvic tumour and resection of the umbilical metastasis were performed. The post-operative macroscopic residual tumour was less than 0.5 cm.

 

III. Histopathologic findings

Macroscopically, both ovaries were unremarkable. The sigmoid tumour mass was 110x95x60mm. The rectal nodule measures 35x23x18mm. The bladder nodule measured 10mm and the pelvic node specimen 40x23 mm.

On microscopic examination, the left ovary showed no evidence of malignancy. No sarcoma was seen in the ovary. Within the atrophic cortex of the right ovary, there was a 3.5mm diameter deposit of adenocarcinoma.

The carcinomatous component included serous carcinoma and squamous carcinoma, with an associated foreign body giant cell reaction to free keratin. The carcinoma was present with poorly differentiated sarcomatous stroma. Malignant cartilaginous nodules with focal ossification were scattered throughout the sarcomatous stroma in keeping with heterologous sarcomatous differentiation (Figure 1). The distribution of disease was primarily peritoneal and very minimal involvement of the surface of only the right adnexa was consistent with a primary peritoneal carcinosarcoma (Figure 2).

Mrs W.B. completed another three cycles of carboplatin and paclitaxel. Currently she is asymptomatic and her CA125 is 9 U/ml.

 

IV. Discussion

We report a case of primary peritoneal MMMT who responded to paclitaxel-based chemotherapy.

Primary peritoneal MMMT is extremely rare. So far, approximately 30 cases of primary peritoneal MMMT have been reported in the literature with the majority of patients being postmenopausal. Compared to other tumours in the female genital tract, primary peritoneal MMMT holds an extremely poor prognosis with most of the patients dying of their disease in less than twelve months (Garamvoelgyi, 1994).

For the present case, the primary therapy was chosen based on the clinical presentation, levels of CA125, imaging results and cytopathologic findings. The assumed diagnosis was advanced epithelial ovarian cancer. Due to the patientÕs age and co-morbidities treatment commenced with single agent carboplatin chemotherapy. However, the CA125 levels increased and symptomatically the patient did not improve. paclitaxel was added to carboplatin for five more cycles. During this treatment the CA125 levels substantially decreased and the patientÕs symptoms improved dramatically. Subsequent surgical debulking revealed the diagnosis of a primary peritoneal MMMT. Cytoreduction was successfully performed.


 

 

Figure 1. The peritoneal MMMT. Section shows the sarcomatous and carcinomatous components

 

Figure 2. Absence of disease in the ovary

 

Ovarian and primary peritoneal MMMT are extremely rare tumours and their prognosis is poor when compared to epithelial cancers (Barnholtz-Sloan et al, 2004). Traditionally, patients with advanced or recurrent uterine MMMT receive ifosfamide, cisplatin and/or doxorubicin. Treatment with single agent ifosfamide produced five complete and four partial responses among 28 patients with advanced uterine MMMT (Sutton et al, 1989). Recently, a GOG study showed a moderate 18% total response rate to single-agent paclitaxel in 44 patients with recurrent or advanced uterine carcinosarcoma who had radiotherapy previously (Curtin et al, 2001). An EORTC phase II study reported an overall response rate of 56% and a median survival of 26 months in a cohort of 48 patients with genital carcinosarcoma treated with concomitant doxorubicin, cisplatin and ifosfamide (van Rijswijk et al, 2003).

The aim of this case report is to share our experience, in which the addition of paclitaxel to carboplatin was crucial to both, improve the symptoms and minimise the disease. Our neoadjuvant chemotherapy approach made optimal surgical debulking possible. Almost one year after treatment, our patient is asymptomatic and well. This case also should encourage investigators to evaluate the role of paclitaxel as part of combination chemotherapy of MMMT.

 

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