Primary Mucinous Cystic Neoplasm of the Retroperitoneum - a Report of Three Cases
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대한병리학회지: 제 37 권제3 호 2003 The Korean Journal of Pathology. 2003; 37: 204-9 Primary Mucinous Cystic Neoplasm of the Retroperitoneum - A Report of Three Cases - Dong Eun Song∙Mi-Jung Kim Primary mucinous cystic neoplasms of the retroperitoneum in women are rare and show his- Shin Kwang Khang∙Eunsil Yu tologic features similar to their ovarian or pancreatic counterparts. We present three cases of Kyung-Ja Cho primary retroperitoneal mucinous neoplasms of variable malignant potentials including a cys- tadenocarcinoma in a 50-year-old woman, a cystic neoplasm of borderline malignancy in a 31- Department of Pathology, University of year-old woman, and a cystadenoma in a 67-year-old woman. All cases showed histologic Ulsan College of Medicine, Asan features similar to mucinous tumors of the ovary. Immunohistochemical study demonstrated Medical Center, Seoul, Korea positivity for calretinin in case 3. The histogenesis of these neoplasms can be assumed most likely to be mucinous metaplasia of the mesothelium or undetected heterotopic pancreatic tissue. Received : August 22, 2002 Accepted : April 22, 2003 Appropriate management is thought to be the same as for analogous ovarian neoplasms. Corresponding Author Kyung-Ja Cho, M.D. Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-dong, Songpa-gu, Seoul 138-736, Korea Tel: 02-3010-4545 Fax: 02-472-7898 E-mail: [email protected] Key Words : Retroperitoneum, Primary Mucinous Cystic Naeoplasm The primary mucinous cystic neoplasm of the retroperitoneum was discovered in the retroperitoneum below the left kidney. On is rare. Only 29 cases have been reported in literature to date, in- abdominal and pelvic computed tomography (CT) with contrast, cluding 2 cases in domestic literature.1-20 They encompass benign, this tumor was found to be located around the pancreatic tail por- borderline and malignant varieties and show a histologic simi- tion and had lobulating contour (Fig. 1). The laboratory data sh- larity to the ovarian mucinous tumors. Although several theories owed a slight elevation of CA-125 (41 UI/mL) and marked ele- have been proposed, the histogenesis of this neoplasm is still uncer- vation of CA 19-9 (1,510 UI/mL). The levels of CEA (4.7 ng/mL) tain. Mucinous or coelomic metaplasia of the retroperitoneal me- and -fetoprotein (1.4 ng/mL) were within normal limits. The sothelium, proposed by Lauchlan,5,7 has gained wide acceptance. cystic mass was located in the mesentery, and no anatomical con- We report three cases of primary retroperitoneal mucinous cys- nections with the pancreas including the tail portion, ovaries, or tic neoplasm ranging the spectrum from benign to malignant small bowel were found. It was initially intact and was removed histologic features, and discuss the histogenesis and appropriate by laparoscopic procedure after the aspiration of the cyst content treatment for these rare neoplasms. to reduce the tumor volume. Grossly, the tumor was a lobulated multiseptate cystic mass (10×9×5 cm and 238 gm), filled with white to yellow thick CASE 1 mucoid material (Fig. 2). The outer surface was grayish tan and glistening, and showed a fibrous adhesion site. The inner surface A 50-year-old woman was admitted to our hospital. She com- was pinkish tan, glistening, and irregular with frond-like areas plained of a palpable abdominal mass in the left upper quadrant and a few solid mural nodules. that she’d had for one month. The previous routine gynecologi- Microscopically, the tumor was composed of irregular multi- cal examination had revealed no abnormalities. On ultrasonog- locular cysts lined by columnar cells with mucinous or eosinophilic raphy, a huge multiseptate cystic mass with a few mural nodules cytoplasm, which were flat or piled up with micropapillary pro- 204 Primary Mucinous Cystic Neoplasm of the Retroperitoneum 205 Fig. 1. Abdominal computed tomography of Case 1 shows a lob- Fig. 2. Gross photograph of Case 1 shows a multiseptate cystic ulated cystic tumor in the left side of the retroperitoneum. mass (10×9×5 cm) with pinkish tan, glistening, and irregular inner surface. trusions showing benign to borderline appearances (Fig. 3A). The the previous operation site, and the remaining solid organs showed adenocarcinoma components were well differentiated with a crib- no metastasis. The patient received chemotherapy using Taxol, riform pattern (Fig. 3B). The stroma consisted of hypocellular but expired ten months after the operation. fibrous or cellular ovarian stroma-like tissue. There were a few anaplastic mural nodules consisting of spindle or multinucleated cells with malignant features, including marked cellular pleomor- CASE 2 phism and high mitotic activity, mixed with mucinous glandu- lar components (Fig. 3C). Some tumor cells lining the cyst showed A 31-year-old woman was admitted to our hospital. She com- strong positivity for Alcian-blue and periodic acid schiff (PAS) plained of abdominal distension in the right lower quadrant that stainings, but negativity for calretinin by immunohistochemi- she’d had for one year. On abdominal and pelvic computed tomog- cal staining. The only mucinous glandular components in the raphy with contrast, a multiseptate cystic mass with calcifications anaplastic mural nodules showed strong positivity for cytoker- was detected in the right retroperitoneum. The laboratory data atin by immunohistochemical staining (Fig. 3D). This tumor was for CA 72-4 in serum was within the normal limit (1.9 /mL). diagnosed to be a primary retroperitoneal mucinous cystadeno- At excision, using a mid-line incision, a well-encapsulated, soft carcinoma. and glistening cystic mass was found in the right retroperitoneal After the laparoscopic operation, the level of CA 19-9 decreased space behind the ascending colon. The mass was easily separat- to 26.5 IU/mL at the two months follow-up, but re-increased to ed from the ascending colon without adhesion. Other solid organs 41.9 IU/mL at the four months follow-up. It increased up to 549 including both ovaries were intact. Incidental appendectomy was IU/mL at the ten months follow-up. The follow-up CT at post- also performed. operative six and eight months revealed neither local recurrence Grossly, the tumor was a smooth-surfaced, multilocular cyst nor distant metastasis despite increased levels of CA 19-9. But, (21×16×10 cm and 210 gm), filled with grayish green tur- it revealed thickening of the walls of the cecum and ascending bid fluid. The inner surface was grayish tan and glistening, and colon with pericolic fat infiltration: both paracolic gutters had showed calcified septa without any solid nodules. thickened and there was a presence of multiple mesenteric lym- Microscopically, the cyst was mostly lined by single layered phadenopathy at the ten months follow-up, which were sugges- low cuboidal or tall columnar cells with mucinous or eosinophilic tive of peritoneal seeding. No recurred mass was identified around cytoplasm (Fig. 4A). In addition, small papillae or glands com- 206 Dong Eun Song∙Mi-Jung Kim∙Shin Kwang Khang, et al. A B Fig. 3. (A) The multilocular cystic wall is lined by mucinous colum- nar cells, which are flat or piled up with micropapillary protrusions. (B) Well differentiated adenocarcinoma components are present in the cystic wall. (C) The mural nodule consists of anaplastic spin- dle or multinucleated cells and is mixed with mucinous glandular components. (D) The mucinous glandular components in the mural C D nodule show positive immunoreactivity for cytokeratin. posed of stratified hyperchromatic nuclei were occasionally ob- CASE 3 served (Fig. 4B). The stroma consisted of cellular ovarian stroma- like tissue or collagenous tissue with multifocal calcifications. A 67-year-old woman was admitted to our hospital. She com- Obvious malignant features or stromal invasion were not present. plained of abdominal discomfort in the right upper quadrant that Immunohistochemistry showed a negative reaction for calretinin she’s had for several months. On abdominal and pelvic comput- in the nucleus and cytoplasm of low cuboidal cells. This tumor ed tomography with contrast, a 5.1 cm sized cystic mass with was diagnosed to be a primary retroperitoneal mucinous cystic homogeneous low attenuation was detected in the posterior lat- neoplasm of borderline malignancy. The appendix was normal. eral side of the ascending colon. There was no lymphadenopa- The post-operative gynecological examination revealed no abnor- thy and the liver showed a tiny nodule in segment VIII, which malities. was too small to characterize. The laboratory data for CEA in serum was within the normal limit (1.9 ng/mL). At laparotomy, Primary Mucinous Cystic Neoplasm of the Retroperitoneum 207 A B Fig. 4. (A) The cystic wall is lined by low cuboidal or tall columnar cells. (B) Borderline malignancy foci show moderate nuclear atypia, strat- ification, and ovarian-like stroma. low cuboidal or tall columnar cells with mucinous cytoplasm (Fig. 5A). The tumor cells showed no evidence of stratification, pleomorphism, or papillary arrangement. The stroma consisted of hypocellular collagenous tissue with multifocal calcifications. PAS and alcian-blue stainings showed a strong positive reaction in the cytoplasm of tall columnar cells. Immunohistochemistry showed positive reaction for calretinin in the nucleus and cyto- A plasm of some low cuboidal cells (Fig. 5B). This tumor was diag- nosed to be a primary retroperitoneal mucinous cystadenoma. The patient was lost to follow-up 3 months after the operation. DISCUSSION Primary retroperitoneal mucinous cystic neoplasms in women are rare. There have been 29 cases reported to date.1-20 This neo- B plasm covers the spectrum from benign to malignant, and a case Fig. 5. (A) The cystic wall is lined by low cuboidal or tall columnar with distant metastasis was reported by Banerjee and Gough.1 cells and hypocellular collagenous stroma. (B) The lining low cu- The origin of this rare neoplasm has been a matter of debate boidal cells show positive immunoreactivity for calretinin.