Tech Coloproctol (2003) 7:55–57
© Springer-Verlag 2003
C A S E R E P O RT
F. Puccio • M. Solazzo • P. Marcianò • R. Fadani • P. Regina • F. Benzi
Primary retrorectal adenocarcinoma: report of a case
Received: 29 January 2002 / Accepted: 20 November 2002
Abstract Primary adenocarcinomas of the retrorectal (presacral) space are uncommon and usually arise from cystic lesions developing from remnants of the embryological postanal gut (tail gut cysts) containing mucous-secreting epithelium. A singular case of a patient who presented with a retrorectal mass is described. A 70–year-old previously healthy man had a four-month history of perianal pain during defecation. Preoperative colonoscopy showed external compression of the lower rectum with normal mucosa; pelvic computed tomography demonstrated a 5.5x4.5 cm2 retrorectal oval mass, originating from the posterior lower rectal wall, containing cystic components and some small calcifications. The tumor, after complete resection via transanal approach, was shown to be a very rare case of primary adenocarcinoma of the presacral space. The patient had an uneventful postoperative course and he is free from disease 4 years after surgery. Clinicopathological features and surgical treatment of this unusual tumor are discussed.
Introduction Adenocarcinomas of the retrorectal space are extremely uncommon. The retrorectal, or presacral, space (Fig. 1) is located between the rectum (upper two-thirds) anteriorly and the sacral promontory and sacrum posteriorly. In this area, a wide spectrum of tumors can be found, including congenital neoplasms and cystic lesions [1–4]. Primary adenocarcinomas arising in this region probably derive from cystic lesions of embryologic origin, remnants of the postanal gut (tailgut cysts) [5, 6]. Clinical diagnosis is usually delayed by non-specific symptomatology and histologic diagnosis is generally obtained after an open biopsy or major operation. Finally, definitive surgical excision is made difficult by anatomic inaccessibility that gives rise to controversy about the choice of an anterior, posterior, transanal or combined operative approach.
Key words Presacral space • Primary retrorectal adenocarcinoma • Transanal resection Case report
F. Puccio • M. Solazzo () • P. Marcianò • R. Fadani • P. Regina F. Benzi Coloproctology Unit of Manerbio Second Surgical Division Manerbio Hospital Via Marconi 7, I-25025 Manerbio (BS), Italy E-mai:
[email protected]
A 70-year-old previously healthy man presented with a four-month history of sacrococcygeal pain during defecation. Digital rectal examination revealed a large, smooth presacral mass. Colonoscopy revealed external compression of the lower rectum with normal mucosa. Transanal ultrasound showed a 6-cm cystic lesion with external compression of the posterior wall of the lower rectum, with no signs of invasion of the prostatic side of the rectal wall. Pelvic computed tomography (Fig. 2) demonstrated a 5.5x4.5 cm2 retrorectal oval mass, originating from the posterior lower rectal wall, containing cystic components and some small calcifications in its anterior left side. Preoperative serum level of carcinoembryonic antigen was 7.7 ng/ml (normal range, 0–5 ng/ml). Histologic examination of the cystic contents after transanal needle aspiration
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F. Puccio et al.: Primary retrorectal adenocarcinoma: report of a case
Fig. 1 Lateral view of pelvic region showing the presacral space
Fig. 2 Pelvic CT scan showing a retrorectal mass containing cystic components and small calcifications in its anterior left side (arrow)
showed absence of tumor cells and presence of numerous leucocytes, histiocytes and cholesterol crystals. The patient underwent surgery and the mass was completely dissected via transanal approach. Intraoperatively there was no evidence of invasion of the rectum, sacrum or
coccyx. Frozen section histologic examination of the specimen disclosed moderately differentiated adenocarcinoma infiltrating the submucosa of the mass (Fig. 3). The tumor contained large amount of necrotic material, high mitotic index (>20 mitoses x 10 HP), a wide area of chronic
F. Puccio et al.: Primary retrorectal adenocarcinoma: report of a case
Fig. 3 Histopathologic analysis of the tumor
inflammation with histiocytosis and a foreign body granulomatous reaction (cholesterol crystals). There was no evidence of lymphatic or hematic invasion by the tumor. Specimen margins were free of tumor. The postoperative course was uneventful. After potential tumoral sources such as gastrointestinal tract, bone and pancreas were eliminated (the patient underwent gastroscopy, colonoscopy and CT of the thorax and the abdomen), the diagnosis of primary retrorectal adenocarcinoma was confirmed. The patient was referred for combined chemotherapy and radiotherapy 2 months after surgery. He completed adjuvant chemo- and radiotherapy two years after surgery. The patient received 5-fluorouracil with folinic acid in combination with local radiation (total dose, 45 Gy). He has had an extensive clinical and radiological evaluation for the last four years and is now free of disease.
Discussion Malignant tumors of the presacral space are uncommon. In all series, chordomas are the most common primary malignant tumors, followed by leiomyosarcomas, neuroblastomas, ganglioneuroblatomas, chondrosarcomas, hemangiopericytomas, teratocarcinomas, squamous-cell carcinomas and adenocarcinomas. While most of the benign presacral tumors are found during infancy and are often asymptomatic, the majority of malignant tumors are discovered in adults and
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usually produce sacrococcygeal pain during defecation and pelvic neurologic dysfunction. Primary retrorectal adenocarcinomas are extremely rare since only a few cases have been reported in the literature. We have not found a report of the same entity in the Italian literature. Usually, retrorectal adenocarcinomas arise from cystic lesions developing from remnants of the embryological postanal gut (tail gut cysts) containing mucous-secreting epithelium. Rarely, they can develop from rectal duplication cysts, a very rare congenital anomaly with only a few cases described in adults [7–9]. Preoperative evaluation consists of physical examination, colonoscopy, transanal ultrasound and expecially pelvic CT that provides the exact location of the lesion and gives some information about the possible nature of the tumor. The surgical approach must be determined by the size and the exact location of the lesion. A small tumor whose proximal end can be palpated by digital examination and that is not invading neighboring structures can be removed through a transanal, transperineal or transcoccygeal approach. For large and high retrorectal tumors, the abdominal approach is mandatory, in order to have control of vital structures such as bladder and ureters and to perform early ligation of the middle sacral vessels. In presence of an extensive neoplasm, a combined abdominal followed by transsacral approach is preferable.
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