Primary retroperitoneal carcinosarcoma in a child: a case report
© Xu et al; licensee BioMed Central Ltd. 2010
Received: 27 March 2010
Accepted: 18 November 2010
Published: 18 November 2010
Carcinosarcoma is a rare biphasic malignancy consisting of intermixed epithelial and mesenchymal elements. Carcinosarcoma is particularly rare among children. We accepted a 7 year old patient with retroperitoneal carcinosarcoma. The tumor was totally resected and no recurrence is found 11 months after operation. Literatures has been reviewed and there are few reports of primary retroperitoneal carcinosarcoma in children up to date. So we report the patient's clinical character, surgical resection, pathological and immunohistochemical analysis.
Carcinosarcoma is a rare malignancy consisting of intermixed epithelial (carcinoma) and mesenchymal (sarcoma) elements, mostly arising from glands and female reproductive system [1, 2]. Carcinosarcoma is particularly rare among children. There are few reports of primary retroperitoneal carcinosarcoma of children in english literatures up to date. We report our experience in treating an unusual case of retroperitoneal carcinosarcoma in a 7 year old patient, its clinical characteristics, surgical resection, pathological and immunohistochemical analysis.
The patient had an uneventful recovery following the surgery and was discharged home on postoperative day 7. On 11 months follow up, the patient did well and gained weight. She was symptom free with normal renal and hepatic function. There was no tumor recurrence was found on CT scan
Primary tumors arising from retroperitoneal space are rare, most are soft tissue tumors. The incidence of retroperitoneal malignancies (mainly sarcoma, such as leiomyosarcoma, fibrosarcoma) is reported as 2.7/10^6 and did not change significantly over the years [3, 4]. Carcinosarcoma is very rare, usually occurs in gland tissue and female reproductive system. However, there is no report of primary retroperitoneal carcinosarcoma so far, especially among children.
Carcinosarcoma consists of mixed epithelial carcinoma and mesenchymal sarcoma components. The ratio of these two components may vary, however, they are related and share many same biological characters . There have been many hypotheses regarding the origin of carcinosarcoma with main dispute on whether the two components of the tumor are homogeneous. Meryer  first classified carcinosarcoma into three types. Type I Collision tumor: the two related carcinoma and sarcoma happen to occur and fuse at the common boarder and infiltrate each other to form a single tumor. Type II Combination tumor: The two type of cell lines in carcinosarcoma derive from the same pleuripotent stem cells to form a single tumor. Type III Composition tumor, in which both components derive from the same tissue concomitantly. Peterson's study  supported that the carcinosarcoma was developed from one same non-specified stem cell line proliferating along both epithelial and metodermal cell lines. Eventually these two related cells formed a single tumor. Recently, most studies agree that carcinosarcoma origins from monoclonal stem cells, rather than multiclonal stem cells .
Carcinosarcoma has no specific clinical symptoms or signs. There is also no specific laboratory and imaging study reported. It is difficult to differ it from other retroperitoneal malignancies before surgical resection. The diagnosis of carcinosarcoma is thus mainly by pathological and immunohistochemical analysis. Most primary retroperitoneal tumors are found due to abdominal pain or abdominal mass. Ultrasound and CT imaging studies can help delineate tumor location and anatomy in relation to adjacent organs in order to make a treatment plan. Final diagnosis is usually immunohistological. H & E staining can clearly show characteristic epithelial and mesenchymal cell components and their differentiation degree. Immunohistological study can determine the tumor components by specific labeling techniques. Most used labeling techniques include vimentin, VI, CD99 for mesenchymal tissue and epithelial membrane antigen (EMA) and cytokeratins for epithelial tissue [5, 9, 10]. Our patient presented with abdominal pain and retroperitoneal mass with unknown origin. Final diagnosis of carcinosarcoma was confirmed by H & E staining and immunohistochemical analysis showing Vimentin (+), Cytokeratins (+) and EMA (+). This also excluded sarcomatoid carcinoma. Sarcomatoid carcinoma is one kind of carcinoma which comes from epithelial cell. The sarcoma-like tissue differentiates from epithelial cell with expression of mesenchymal markers. So is not a true sarcoma or carcinosarcoma .
Carcinosarcoma is a highly malignant and invasive tumor. Retroperitonal carcinosarcoma can often occur insidiously without obvious signs or symptoms until the tumor grow large enough to compress adjacent organs. Currently, surgical resection is still the main and most effective treatment method [11, 5]. Surgery requires complete resection of the tumor together with infiltrated adjacent organ tissue. It has been reported that the prognosis depended on the negative surgical margin [12, 13]. Adjuvant chemotherapy may not be satisfactory in some patients, but many studies showed that combined surgery and chemotherapy or radiotherapy can often achieve a much better prognosis, especially among children. This is due to: 1. Retroperitoneal tumor is usually very large upon discovery with many complex adjacent organ tissue infiltration making complete surgical resection a challenge. So it is not uncommon that residual tumor or positive surgical margin occur more often than tumors of other locations. Mendenhall  summarized several large patient population studies and found that only 50-67% of retroperitoneal sarcoma achieved complete surgical resection. Thus, combined chemotherapy or radiotherapy are often used to compensate and hope to reduce postoperative tumor reoccurrence [15, 16]. 2. In contrast with adult, sarcoma in children are mainly composed of striated muscle sarcoma and fibrosarcoma, which are usually sensitive to chemotherapy and radiotherapy. So chemotherapy and radiotherapy often improved survival rate of sarcoma in children . In our case, the tumor and the involved right kidney were completely removed with negative surgical margin. With only one kidney remaining in a 7 year old child, we did not employ chemotherapy or radiotherapy postoperatively so as to avoid possible severe complications. We follow the patient closely, she is doing very well 11 months after the surgery without tumor recurrence was found.
Currently, there has been no prognosis study on primary retroperitoneal carcinosarcoma. However, inferring from prognosis study of carcinosarcoma in other locations, prognosis of retroperitoneal carcinosarcoma can be very poor, given its highly malignant, rapid aggressive infiltration and easy postoperative metastatic nature [11, 17].
Carcinosarcoma is a rare biphasic tumor with high malignancy, aggressive invasiveness and poor prognosis. Preoperative diagnosis is often difficult, especially for retroperitoneal carcinosarcoma, due to its insidious onset and lack of specific laboratory and imaging study available. Confirming diagnosis requires immunohistological studies. Surgical resection is still the most important treatment, plus adjuvant chemotherapy and possible targeted therapy could improve survival rate. However, satisfactory result is still hard to achieve. This is especially the case among children due to the severe complication from chemotherapy limiting it application in these patients. The prognosis of our patient could be get depending on further follow ups.
Written informed consent was obtained from the patient's father for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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