A rare case of primary extragenital retroperitoneal carcinosarcoma with review of the literature
© Springer-Verlag 2009
Received: 7 November 2008
Accepted: 29 December 2008
Published: 15 January 2009
Carcinosarcomas are rare, highly aggressive neoplasms that most commonly arise from the female genital tract but can unusually present in extragenital locations. To the best of our knowledge, only three cases of primary retroperitoneal, extragenital carcinosarcomas have been documented in the English literature to date. A 72-year-old woman presented with onset of abdominal pain and urinary retention. Computed tomography scan revealed a mass in the retroperitoneal space with hydronephrosis and partial obstruction of the left ureter. Lost to follow-up at that time, the patient returned 6 months later with a left leg deep vein thrombosis. On exploratory laparotomy at that time, the retroperitoneal mass was found to completely engulf the left ureter, iliac artery, and vein. Resection was not possible, but biopsy confirmed the presence of an extragenital carcinosarcoma.
A 72-year-old Caucasian female presented with new onset left-sided abdominal pain and urinary retention. The patient’s medical history was insignificant except for remote total abdominal hysterectomy and bilateral salpingoophorectomy done for symptomatic fibroids.
During exploratory laparotomy, the mass was found in the lower left retroperitoneal space with extension up to the aorta and was deemed unresectable. A 3- × 2-cm open tissue biopsy was obtained, and pathology confirmed the presence of extragenital carcinosarcoma.
Pathology: gross and microscopic
The needle biopsy of the retroperitoneal mass grossly consisted of multiple light tan, cylindrical fragments of soft tissue. Microscopically, the biopsy showed an infiltrative malignant neoplasm consisting of a glandular epithelial component and a stromal spindle cell component. There was a background of collagenous fibrosis and focal necrosis.
Three cases reporting primary retroperitoneal carcinosarcoma
Ferrie and Ross 
Herman and Tessler 
Shintaku and Matsumoto et al. 
Weakness, anorexia and vague abdominal pain
Abdominal fullness, pain, diarrhea and nausea
11 cm diameter, 650 g
20 cm diameter, 1,040 g
En bloc resection
Debulking with left nephrectomy
En bloc resection with TAH-BSO and omentectomy.
Retroperitoneum below right kidney
Retroperitoneum involving the left kidney and ureter
Left retroperitoneum extending to lateral pelvic wall
Adriamycin, Cytoxan, DTIC, Vincristine
Intraperitoneal carboplatin then adjuvant epirubicin hydrochloride and carboplatin
Bilateral pulmonary nodules
Malignant stroma of endometrial type
Carcinosarcomas are classified by what type of sarcomatous tissue is present. Two types have been described: homologous type if a sarcoma is of uterine origin and heterologous type if extra-uterine tissue is present, such as muscle, cartilage, or bone . Heterologous mixed mullerian tumors carry a worse prognosis than homologous. It is thought that the ability of the tumor to form heterologous tissue types denotes more aggressive potential .
The origin of extragenital mullerian tumors is uncertain. An association with radiation has been suggested , but lack of firm evidence for this correlation has been discounted by some authors . Three main hypotheses have been proposed. First, these malignancies may arise out of ectopic foci of endometriosis. Although there have been reports of carcinosarcomas arising from endometriosis , there are other cases in which no endometrial tissue was able to be documented . No endometrial tissue was demonstrated in our case. Another hypothesis features the so-called secondary mullerian system . The secondary mullerian system, first described by Lauchlan, posits that the abdominal and pelvic peritoneum and retroperitoneal mesenchyme retain the potential for mullerian differentiation, probably due to their close embryologic relation to the mullerian ducts [7, 8]. Therefore, these tissues of the secondary mullerian system could possibly spontaneously differentiate into mullerian malignancies. Lastly, mullerian carcinosarcomas could begin as poorly differentiated epithelial neoplasms that undergo transformation into mesenchymal differentiation . The transformation of carcinomas into tumors with sarcomatoid elements has been previously demonstrated in ovarian carcinomas .
Most extragenital carcinosarcomas reported have occurred in middle-aged to older individuals . The CA-125 tumor marker was noted to be elevated in at least one case and returned to normal after the tumor was removed . However, although CA-125 can be elevated when the carcinomatous element of the carcinosarcoma is predominating, it is not an effective diagnostic tool and is useful primarily for follow-up . The prognosis for extragenital carcinosarcomas is poor, with most patients dying within 1 year [3, 6]. Knowledge of the most effective treatment is limited due to the paucity of cases , and chemotherapy and radiation are of limited effectiveness . However, carboplatin–paclitaxel has been shown to be effective against uterine MMMT  and may offer some benefit in tumors of this type as well. Surgical resection remains the mainstay of treatment, even if the goal of therapy is palliation. Given the propensity of extragenital carcinosarcomas for invasion of surrounding structures, it is recommended that tumor removal is performed by an oncologic surgery team, often involving members of different surgical subspecialities.
Carcinosarcomas are rare tumors that histologically contain the epithelial and mesenchymal elements of both a carcinoma and a sarcoma. Although rare, a high level of suspicion must be maintained for any fast-growing tumor of the retroperitoneal space. Found primarily in the female reproductive tract, carcinosarcomas can arise from a variety of extragenital locations including the retroperitoneal space. Surgical excision is the treatment of choice in cases amenable to resection. Early detection and aggressive surgical intervention should be the goal of any clinician faced with this rare but aggressive neoplasm.
Conflict of interest
The authors declare that there are no conflicts of interest.
The authors declare that no financial support was received for this article.
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