Peritoneal papillary serous carcinoma is a rare tumor that involves the surface of the pelvic and/or abdominal peritoneum. Long-term survival among patients with this tumor has been rare. Most patients with this cancer have been treated with debulking surgery and postoperative chemotherapy. A case of incompletely resected peritoneal papillary serous carcinoma with a complete response to cisplatin-based chemotherapy is reported. Subsequent laparotomy revealed no residual tumor. This case suggests that primary chemotherapy may be successful in treating unresectable primary papillary serous tumors of the peritoneum

Kim, Wooshin

Saleh, Walid A.

Van de Ven, Cosmas J. M.

English

Henry Ford Health System Scholarly Commons

Henry Ford Hospital Medical Journal Volume 40 Number 1 Article 32 3-1992 Primary Papillary Serous Carcinoma of the Peritoneum: A Case of Complete Remission of Bulky Peritoneal Disease After Chemotherapy Walid A. Saleh Cosmas J. M. Van de Ven Wooshin Kim Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Saleh, Walid A.; Van de Ven, Cosmas J. M.; and Kim, Wooshin (1992) "Primary Papillary Serous Carcinoma of the Peritoneum: A Case of Complete Remission of Bulky Peritoneal Disease After Chemotherapy," Henry Ford Hospital Medical Journal : Vol. 40 : No. 1 , 136-138. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol40/iss1/32 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Primary Papillary Serous Carcinoma of the Peritoneum: A Case of Complete Remission of Bulky Peritoneal Disease After Chemotherapy Walid A. Saleh, MD,* Cosmas J. M. Van de Ven, MD,* and Wooshin Kim, MD' Peritoneal papillary serous carcinoma is a rare tumor that involves ihe surface ofthe pelvic andior abdominal peritoneum. Long-ierm survival among patients with this tumor has been rare. Most patients with this cancer have been treated with debulking surgeiy and postoperative chemotherapy. A case of incompletely resected peritoneal papillary serous carcinoma with a complete response to cisplatin-based chemotherapy is reported. Subsequent laparotomy revealed no residual tumor. This case suggests that primary chemotherapy may be successful in treating unresectable primary papillary serous tumors ofthe peritoneum. (Henry Ford Hosp MedJ 1992;40:136-8) Papillary serous carcinoma (PSC) of the peritoneum was first described by Swerdlow (1) in 1959. It involves diffusely the peritoneum, histologically resembles PSC of the ovary, but ei-ther spares the surface of the ovary or involves it only micro-scopically (2). About 130 cases have been described under a va-riety of names including mesotheliomas, mesodermoma, perito-neal PSC, papillary carcinoma from extraovarian tissue, serous surface papillary carcinoma of the ovary, and peritoneal car-cinomatosis of unknown primary site. There is still no consen-sus regarding nomenclature, histogenesis, immunohistochemi-cal characteristics, clinical behavior, and treatment. Case Report A 75-year-old white female, gravida 3, para 3, underwent surgery in a local hospital for possible incarcerated inguinal hernia. There was a four-month history of gradually increasing abdominal girth, and on ad-mission her abdomen was markedly distended. She denied any change in weight. Physical examination confirmed the presence of abdominal distension and tympany and hyperactive bowel sounds and disclosed a 3 cm ovoid nontender, nonreducible mass in the right inguinal area. On admission, x-rays and laboratory tests were reportedly normal. With the patient under spinal anesthesia, a right incarcerated femoral hernia sac was opened. During exploration, muhiple polypoid implants sug-gestive of carcinomatosis along with a large amount of brown-colored peritoneal fluid and necrotic tumor material were encountered. There was no evidence of bowel in the hernia. The incision was extended and abdominal exploration revealed marked ascites, diffuse tumor infiltra-tion of the entire pelvis and small bowel, and "caking" of the lower third of the omentum. The liver and upper abdominal structures were not involved, and there was no evidence of bowel obstruction. After multiple biopsies were taken from the omentum, hernial sac, and pelvic peritoneum (Figure), the incision was closed as the tumor was consid-ered to be unresectable. The postoperative course was uneventful ex-cept for mild ileus. On the recommendation of the medical oncologist, the patient received nine courses of Cytoxan and carboplatin over a total of eight months following the usual protocol for ovarian carcinoma. Follow-up visits revealed that the patient was doing well with no clini-cal evidence of disease. The carcinoembryonic antigen (CEA) and CA 125 levels were consistently normal. After completion of the chemo-therapy, abdominal computed tomography (CT) revealed a 1 cm soft tissue density at the level of the cecum, which was unchanged from a previous CT one month earlier. The patient was referred to Henry Ford Hospital for a second explor-atory laparotomy 11 months after the original surgery. She underwent lysis of adhesions, cytology washings, total abdominal hysterectomy and bilateral salpingo-oophorectomy, selective pelvic and paraaortic lymph node dissection, omentectomy, and repair of right inguinal her-nia. There were dense adhesions around the bowel and adnexae. Both ovaries were atrophic, about 1 cm in greatest diameter. A 2 cm nodule was found in the right pelvic side wall, posterior to the cecum, with multiple small yellowish nodules on the pelvic floor and peritoneal sur-face. Overall there was no gross evidence of disease. The pelvic wash-ings and multiple biopsies were negative. The patient was alive and free of disease 11 months after her second exploration. Discussion Many previous studies indicate that patients with primary PSC have a worse prognosis than do those with ovarian cancer. It is unclear whether this worse prognosis is due to the advanced disease which is frequentiy found in this tumor or to unknown inherent biological characteristics. We present a case of primary PSC and extensive peritoneal involvement with absence of dis-ease surgically documented after chemotherapy. Malignant tumors involving the peritoneum are usually due to metastatic ovarian carcinoma, tn rare cases, no primary tumor Submitted for publication; July 22, 1991. Accepled for publication; December 19, 1991. *Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Henry Ford Hospital. tMatemal-Felal Medicine, Duke University Medical Center, Durham, NC. *Address correspondence to Dr. Saleh, Departmenl of Obstetrics and Gynecology, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202. 136 Henry Ford Hosp Med J—Vol 40, Nos 1 & 2. 1992 Cure of Unresecled Peritoneal Cancer—Saleh et al can be found and a primary tumor of the peritoneum must be considered. Of course other primary tumors must be ruled out. As in ovarian serous tumors, benign, borderiine, and malignant variants of papillary serous tumors have been reported (3). Be-nign or borderline peritoneal tumors must be differentiated mor-phologically from mesotheliomas (4). PSC is frequentiy de-scribed microscopically as showing mitotic figures and many psammoma bodies (5,6). Most PSC tumors have at teast a few celts that show cytoplasmic or surface mucin and stain posi-tively for keratin and epithelial membrane antigen. Other mark-ers such as Leu-Ml, B72.3, vimentin, HPLAP, and CEA are found in a small percentage of cells (6,7). In fact, on the basis of light microscopy and histochemical and immunohistochemical characteristics, PSC cannot be differentiated from ovarian carci-noma. This lack of cytochemical discrimination is explained by the common histogenesis of these neoplasms. Both the perito-neal mesothelium and the surface epithelium of the ovary de-velop from the embryonic coelomic epithelium. The commonly accepted theory for the origin of peritoneal papillary carcinoma is that it arises directly from the mesodermal lining of the ab-dominal cavity in response to an unknown oncogenic stimulus (4,8). On the other hand, some believe that PSC arises from em-bryonic rest cells (5). In any event, the coelomic origin of these tumors explains their resemblance in many respects to ovarian cancer. The fact that PSC has occurred in patients after bilateral salpingo-oophorectomy supports the concept of its peritoneal origin (9). PSC affects only women, whose median age is about 60 years (4,10), Abdominal pain and distension are the most common presenting symptoms (6,9) while a palpable mass is uncommon (4) . Radiological studies including CT are usually not helpful in diagnosis (6). In most cases the entire mesothelial surface is in-volved and the tumor is rarely predominant in or limited to the pelvis. Generally the ovaries are invotved only superficially and are of normal size. When the tumor invades the ovaries, one en-counters superficial involvement as well (6). PSC frequentiy in-volves the submesothelium, but deep invasion into the abdomi-nal or pelvic organs or either local or distant metastases are rare (5) . Spread of the disease outside the abdominal cavity produc-ing malignant pleural effusion has been described (9). Ascitic fluid, when present, usually contains malignant cells. The median survival time of patients with PSC is between 11 and 24 months, simitar to those of the same age group who have ovarian cancer (2,9). Some have found that peritoneal tumor has a worse prognosis than ovarian cancer (11,12). White most se-ries report only a few long-term survivors. Ransom et al (13) de-scribed three patients who lived over six years. Chen and Flam (3) atso reported long-term survival after cisplatin-based che-motherapy. These patients had undergone optimal debulking surgery followed by cytoxan-cisplatin chemotherapy. There is no consensus as to the best therapy for peritoneal car-cinoma, but most authors prefer to treat it in the same manner as ovarian cancer (2,4,9). Complete clinical response to postopera-tive chemotherapy has been reported in 20% to 25% of patients (2,4,6), but not all respond so favorably (13). Patients treated with combination chemotherapy do better than those treated with single agents and cisplatin-based regimens seem to be Figure—Papillary serous carcinoma ofthe peritoneum (hema-toxylin-eosin stain, X50). more effective (4). Contrary to other authors, Fromm et at (4) in their large series of 74 patients found that neither chronologic age nor the presence of residual disease after cytoreduction de-termined prognosis. This finding is in contrast with that found in ovarian cancer. Fromm et al concluded that the absence of mito-ses represented a favorable prognostic factor. Lele et al (2) de-scribed one patient with complete response to cisplatin, bleo-mycin, and cytosine arabinoside administered intraperitoneally. Most studies conceming PSC of the peritoneum are retrospec-tive and there is no staging system. Few reports allow conclu-sions about therapy, most focus on the pathology, and all are ret-rospective in nature. Only prospective studies conducted in multiinstitutional clinical trials can provide reliable data about the natural history of this disease. The vatue of intraperitoneal chemotherapy must be carefully investigated. Our case suggests that chemotherapy may be successful in treating unresectable primary PSC of the peritoneum. Acknowledgment We thank Dr. Amnuay Singhakowinta for his assistance and Philip Dvoretsky for his critical review of the pathology. References 1. Swerdlow M. Mesothelioma of the pelvic peritoneum resembling papillary cystadenocarcinoma of the ovary: Case report. Am J Obstet Gynecol 1959;77: 197-200. 2. Lele SB, Piver MS, Matharu J, Tsukada Y. Peritoneal papillary carcinoma. Gynecol Oncol 1988;31:315-20. 3. Chen KT. Flam MS. Peritoneal papillary serous carcinoma with long-term survival. Cancer 1986;58:1371-3. 4. Fromm G-L, Gershenson DM, Silva EG, Papillary serous carcinoma of the peritoneum. Obstet Gynecol 1990;75:89-95. 5. Kannerstein M, Churg J, McCaughey WTE, Hill DP. Papillary tumors of the peritoneum in women: Mesothelioma or papillary carcinoma. Am J Obstet Gynecol 1977;127:306-14. 6. Tmong LD, Maccato ML, Awah H, Cagle PT, Schwartz MR, Kaplan AL. Serous surface carcinoma of the peritoneum: A clinicopathologic study of 22 cases. Hum Pathol 1990;21:99-110. 7. Raju U, Fine G, Greenawald KA, Ohorodnik JM. Primary papillary serous neoplasia of the peritoneum: A clinicopathologic and ultra.stmctural study of Henry Ford Hosp Med J—Vol 40. Nos I & 2, 1992 Cure of Unresected Peritoneal Cancer—Saleh et al 137 eight cases. Hum Pathol 1989;20:426-36. 8. Parmley TH, Woodruff JD, The ovarian mesothelioma. Am J Obstet Gyne-col 1974;120:234-41. 9. Dalrymple JC, Bannatyne P, Russell P, et al. Extraovarian peritoneal serous papillary carcinoma: A clinicopathologic study of 31 ca.ses. Cancer 1989:64: 110-5. 10. Clement PB. Endometriosis, lesion of the secondary mullerian system and pelvic mesothelial proliferation. In: KurmanRJ.ed. Blaustein's pathology ofthe female genital tract. New York: Springer-Verlag, 1987:438-70. 11. Gooneratne S. Sassone M, Blaustein A, Talerman A. Serous surface papil-lary carcinoma of the ovary: A clinicopathologic study of 16 cases. Int J Gynecol Pathol 1982;1:258-69. 12. Mills SE, Andersen WA, Fechner RE, Au.stin MB. Serous surface papil-lary carcinoma: A clinicopathologic study of 10 cases and comparison with stage III-IV ovarian serous carcinoma. Am J Surg Pathol 1988; 12:827-34. 13. Ransom DT, Patel SR, Keeney GL, Malkasian GD, Edmonson JH, Papil-lary .serous carcinoma of the peritoneum: A review of 33 cases treated with Platin-based chemotherapy. Cancer 1990:66:1091-4, 138 Henry Ford Hosp Med J—Vol 40, Nos I & 2, 1992 Cure of Unresected Peritoneal Cancer—Saleh et al 

Primary Papillary Serous Carcinoma of the Peritoneum: A Case of Complete Remission of Bulky Peritoneal Disease After Chemotherapy

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Primary Papillary Serous Carcinoma of the Peritoneum: A Case of Complete Remission of Bulky Peritoneal Disease After Chemotherapy

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