A Case Report and a Literature Review of Primary Retroperitoneal Mucinous Cystadenoma: The Importance of Imaging in Diagnosis and Management

Abstract

Primary retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare tumor: its histogenesis and its biological behavior remain speculative. Since most retroperitoneal tumors are malignant, a preoperative diagnosis of benignity is essential and it can be reached through imaging examinations, allowing a conservative management approach. We describe the case of a 52-year-old woman with abdominal pain and a palpable mass. Computed tomography of the abdomen revealed a retroperitoneal cystic mass, which was resected successfully through laparoscopy and diagnosed as PRMC. Although there are no pathognomonic, clinical or radiological findings for PRMC, it should be included in the list of differential diagnoses and its imaging criteria of benignity should always be sought, with the aim to exclude malignant tumors.

First Report of Retroperitoneal Mucinous Cystadenoma in a Patient with Hirsutism

“…There are two main hypotheses currently proposed for their origin. 2 The first hypothesis is that these cysts are ovarian in origin, which is supported by the literature and positive expression of ER in the present case ( Figure 3A). 2 Since ER is expressed on cells in both the breast and ovaries, this finding does not definitively mean that the cyst was ovarian in origin, but it supports the first hypothesis.…”

“…definitive diagnosis can only be made through examination of the excised tissue, complete surgical excision is recommended. [1][2][3] While most patients are reported to present with pain or other symptoms (Table 2), our patient had no symptoms directly attributable to the retroperitoneal cystic lesion. The patient's DHEA-S levels, which were elevated to 458 μg/dL at the time of initial surgical consultation, normalized to 300 μg/dL following surgical excision of the cystic lesion.…”

“…While the origin of these cysts is unknown, the strong presence of ER and absence of calretinin on the patient's cyst supports the theory that these cysts arise from the ovaries. 2 Additionally, the presence of ER may provide a future link between PRMC and PCOS. This is the first case report of the observation between a PRMC, elevated levels of androgens, and hirsutism.…”

“…Recent documented reports of laparoscopic excision suggest that minimally invasive resection may be a safe and effective management strategy for this condition. 2,9,10,24,27,31,34,46,47 It is important to note that in a comprehensive review of the English literature, there are 21 cases of borderline malignant mucinous cystadenomas and 80 cases of malignant mucinous cystadenocarcinoma. 9,[53][54][55] The presence of these other categories of cystic lesions supports the need for surgical resection to definitively diagnose and reduce the potential for future cancer development in these lesions.…”

“…rimary retroperitoneal mucinous cystadenomas (PRMC) are rare neoplasms of unknown origin most commonly reported in women of childbearing age. 1 Preoperative diagnosis of benign PRMC is difficult based on radiology findings alone, 2 and limited biopsy or laboratory sampling is insufficient for definitively diagnosing PRMC. 3 Therefore, PRMC is generally diagnosed post-surgical resection.…”