Primary Papillary Serous Carcinoma of the Peritoneum

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Annah ofOncology 12 56.1-567. 2001 © 2001 Kiuwer Acadenm Publishers Printed in the Netherlands

Clinical case Primary papillary serous carcinoma of the peritoneum in a man E. Shmueli,1 L. Leider-Trejo,2 I. Schwartz,2 D. Aderka1 & M. Inbar1 Departments of ^Oncology, 2Pathology, Iclulov Hospital, Sorasky Medical Center, Tel-Aviv, Israel

Summary Background Primary papillary serous carcinoma of the peritoneum is a well-known entity in women The tumour is derived from the extraovanan mesothelium and the pelvis and lower abdomen mesothelia The treatment strategies are similar to ovarian serous papillary carcinoma Patients and methods A case of primary serous papillary carcinoma of the peritoneum in a man is presented The patient, 53 years old, died 2 months after diagnosis Results The histologic and immunohistochemical studies

Case report In another hospital a 53-year-old man, vegetarian, presented with a 4-week history of abdominal pain, changes in bowel habits, weight loss and cough The past history included acute pericarditis at the age of 21, and GuillanBarre syndrome at the age of 33, both resolved without sequelae Physical examination showed abdominal distention, epigastric tenderness and reduced breath sounds in the left lung. Abdominal ultrasound revealed ascites and cholelithiasis. Chest X-ray revealed bilateral pleural effusions. Abdominal and chest CT scans showed ascites, pleural effusions, enlarged para-aortic lymph nodes and a reticulonodular infiltration of the omentum Colonoscopy, gastroscopy and small bowel passage were unremarkable Testicular ultrasound showed left hydrocele without evidence of space-occupying lesion in the testes. Endoscopic ultrasound did not show any pathology in the penampulary region or in the pancreas Bone scan was normal, and bone marrow biopsy showed normal reactive marrow. Bronchoscopy and bronchoalveolar lavage were unremarkable Blood tests showed hypoalbuminemia and thrombocytosis. Kidney and liver function tests were

Key words diagnosis, man, papillary carcinoma, pathology, peritoneal neoplasm

normal. Serum CEA, PSA, aFP, pHCG were within normal limits, CA19.9 was 650 The ascitic fluid, which proved to be an exudate, was tapped twice but no malignant cells could be detected. Examination for acid-fast bacteria was negative. The left pleural effusion was also tapped, but again it did not contain malignant cells The patient was referred to our department The physical examination revealed an enlarged left supraclavicular lymph node. The biopsy revealed metastatic adenocarcinoma with calcifications. Immunohistochemical stains were positive for CK 20 in some cells and CA19.9 in glandular pattern. Stains for polyclonal and monoclonal CEA, S-100 and thyroglobulin were negative A pancreatic or pulmonary origin was suspected The patient received 3 courses of cisplatin 100 mg/m 2 dl and 5-fluorouracil 1000 mg/m" d 1 —4 continuos infusion, but the disease progressed A further histologic and immunohistochemical analysis of the lymph node revealed high-grade adenosquamous carcinoma with glandular component that had distinct papillary configuration. CA125 was positive, pan keratin-positive, PAS-weakly positive and leu-Ml-positive. Despite the treatment the patient suffered from progressive disease, characterized by anasarca, progressive weight loss and recurrent pleural effusions and ascites A change of the treatment to paclitaxel 175 mg/m 2 dl and gemcitabine 1000 mg/m" dl and d4 did not influence disease progression, and two months after diagnosis the patient expired Autopsy showed widespread, sheet-like, gray-white, bulky diffuse tumour masses, with papillary structures

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Primary papillary serous carcinoma of the peritoneum (PSCP) is a clinicopathological entity, which was described exclusively in women. We present an unusual case of PSCP in a man In a review of the medical literature we could find only a single case of PSCP in a man [1], to which we add the present second case.

of the tumour will be presented These studies, made during lifetime and at autopsy of the patient, confirm a diagnosis of primary serous papillary carcinoma of the peritoneum Conclusions primary serous papillary carcinoma of the peritoneum can occur in men, and should be considered in the differential diagnosis in cases of abdominal carcinomatosis of unknown origin Treatment options remain to be determined


Figure I Mucroscopic widespread tumour masses involving peritoneal surface


B Figure 2 Papillary carcinoma (A) micropapillary structures wit some signet ring cell appearance (B)

and cysts formation (Figure 1) The cysts were up to 3 5 cm in diameter and filled with gray partially gelatinous material. The tumour covered the visceral and parietal peritoneal surfaces, and formed a conglomerate of firmly adherent loops on the small and large bowel The serosal and subserosal layers of the bowel wall were infiltrated by the tumour. The tumour also covered the diaphragm, oesophagus, omentum, appendix, kidneys and pleura Metastatic tumour was found in para-aortic, pengastnc and penpancreatic lymph nodes and in the left adrenal

Primary papillary serous carcinoma of the peritoneum was first described by Swerdlaw, 1959, in a 27-year-old female patient [2] Since then, reports in the literature suggested that approximately 10% of women diagnosed with ovarian carcinoma actually have PSCP [3-5]. Some reports indicated a poor prognosis for women presenting with peritoneal carcinomatosis [6-9], but long-term survivors were described after the use of platinum-containmg regimens [7, 10-13]. Piura et al even suggested that when treatment strategies for stage III—IV primary ovarian carcinoma are applied to PSCP, the survival of PSCP patients may be similar to or even better than that of patients with stage III—IV ovarian cancer [14]. In the past, this disease has been reported under many different names including: serous surface papillary carci-

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Multiple intravascular tumoural emboh were found in visceral organs including the heart, brain, liver, spleen, urinary bladder, prostate, testes and seminal vesicles. The body and head of the pancreas with the duodenal region were involved in a matted mass, but there was no recognizable tumour in the pancreas itself. All autopsy specimens were examined with routine haematoxylin and eosin stains. Epithelial mucine was demonstrated by mucicarmin stain (Sigma, Minnesota, USA), alcian blue stain (Bio optica, Milan, Italy) and with periodic acid-Schiff (PAS) reaction before and after diastase digestion. Immunohistochemical procedures were performed using LCA, vimentin, EMA, CK7 (Daco, Glostrup, Denmark), CK20, CA125, CD15 (leuMi), ER, PR (Ventana, Texas, USA), CEA, S-100, cytokeratins AE1 and AE3, and calretinin (Zymed, California, USA) Electron microscopy (EM) was performed from the peritoneal specimen. Histologically, the autopsy material showed a moderately to poorly differentiated carcinoma with micropapillary serous structures (Figure 2a). Some cells with signet-ring appearance and cytoplasmic mucin were noted (Figure 2b). The tumour cells were weakly positive for PAS. Immunohistochemically the tumour cells were strongly positive for EMA, CD15, CK7 (Figure 3a), CK20 and for low and high molecular cytokeratins. CA125 showed focal positivity (Figure 3b). The immunostains for CEA, S-100, ER, PR, vimentin and LCA were negative, and were not contributory for calretinin. EM examination showed single type tumour cells The nucleoli were euchromatic. Numerous microvilh were present on the covering apical surface of the tumour cells (Figure 4) None of the microvilh appeared brushy or branched - thus making a diagnosis of mesothehoma unlikely. There were rare, small, well-formed desmosomes but no tight junctions. Histologically, immunohistochemically and electronmicroscopically the findings confirmed a diagnosis of primary serous papillary carcinoma of the peritoneum.


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Figure 4 Eectron micrograph showing tumour cell with microvilh

Immunihistochemical staining forCK 7 (A) and CA125 (B)

noma, primary peritoneal carcinoma, extraovanan pelvic serous tumour, multifocal extraovanan serous carcinoma, peritoneal carcinomatosis of unknown primary tumour site, and even peritoneal mesothelioma. In 1977, Kannerstein emphasized the distinction between PSCP and peritoneal mesothelioma [15], the former occurring exclusively in women, and the latter predominantly affecting men exposed to asbestos. PSCP is now believed to be a distinct clinicopathologic entity, derived from the extraovanan mesothehum, which has Mullenan potential. The histogenesis of PSCP is not well-described Lauchelan in 1972 described the female peritoneum as a part of the secondary Mullenan system [16]. The surface (germinal) epithelia of the ovary and the mesothelia of the pelvic and lower abdominal peritoneum arise from the same embryonic origin under Miillenan influence. PSCP is not always confined to the pelvis and, moreover, simultaneous cancers of the salpinx and endometrium have been reported [17, 18]. Troung et al suggested that a 'field effect' plays a role in the pathogenesis of PSCP [19]. Since the disease is found almost exclusively in women, a hormonal influence was suspected. Nevertheless, oestrogen and progesterone receptors are not a common finding in PSCP and are expressed in only 30%-50% of cases [20, 21]. Only one case of PSCP in a man has been described [1] During fetal development of the male embryo

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Miillenan ducts do develop but disappear within 9-12 weeks under the influence of Miillenan inhibitory factor (MIF). The fetal testes secrete this factor, which is responsible for inhibiting the development of the Miillerian duct This temporary presence of the Mullenan ducts in the male embryo may account for the rare occurrence of lesions resembling serous papillary ovarian cancer in the adult male patient Patients suffering from PSCP usually present with complaints of abdominal distention, pain or pressure, associated with ascites and gastrointestinal symptoms (loss of appetite, nausea, vomiting, change in bowel habits) [4, 11, 19, 22-24]. On physical examination, the most common finding is ascites The clinical presentation is usually indistinguishable from advanced ovarian cancer. Serum levels of CAI25 are elevated in most PSCP patients in whom values are obtained prior to treatment [8, 22, 23] The differential diagnosis of PSCP in women includes' endosalpingiosis - a benign lesion, found most often in association with chronic salpingitis Borderline tumours of the peritoneum might present a clinical feature similar to PSCP, but has an excellent prognosis [25, 26]. Secondary peritoneal carcinomatosis from other primary tumour sites, such as breast, gastrointestinal tract (especially stomach, pancreas) and lungs must be ruled out. Malignant mesothelioma might be clinically indistinguishable from PSCP, but the former is more frequent in men, and is rare in the absence of asbestos exposure. Isolated malignant mesothelioma of the peritoneun is uncommon, and it usually occurs concomitantly with pleural involvement. In male patients mesothelioma of the tunica vaginalis has been described [27-30]. The presence of cysts may indicate multicystic mesothelioma, a lesion that is more common in women [31] Our patient had no history of asbestos exposure, nevertheless, on autopsy pleural involvement was found. Definite distinction between malignant mesothelioma and PSCP requires a detailed histologic, lmmunohistochemical and electron microscopic work-up [24, 32-34] Histologically, in PSCP cuboidal epithelial cells, characterized by papillary structures, are found in tumour tissue Spindle cells and sarcomatoid components are common, and psammoma bodies might be found [9, 35].


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Ultrastructural examination reveals epithelial differentiation, cytoplasmic mucin, microvilli and occasionally cilia [19]. The need for extensive immunohistochemical work-up came about especially after reports of mucin-producing mesothehoma [36] and elevated serum CA125 in mesothehoma patients [37, 38]. Characteristically, adenocarcinoma stains positive for mucin, cytokeratin, EMA, CA125, CEA, leu-Ml, whereas mesothelioma usually stains positive for vimentin, cytokeratin and EMA. Occasionaly reactivity for CA125 and leu-Ml has been described [24, 32, 33, 39] Histochemical analysis of tumour tissue obtained from our patient demonstrated positive stains for CA125, pan-keratin and leu-Ml, focally positive for PAS, and negative for CEA, S-100, vimentin and thyroglobulin. The ultrastructural findings were different from those of metastatic carcinoma and of malignant mesothelioma, and were conclusive for the diagnosis of primary serous papillary carcinoma of the peritoneum. As in the only published case of PSCP in man [1], our patient suffered from a fulminant and rapidly progressive disease. He did not respond to cisplatin-based regimen, and died soon after one course of pachtaxel and gemcitabine was administered, about two months post diagnosis. Therefore the proper treatment of PSCP in man remains to be determined.

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Received 16 August 2000. accepted 16 October 2000 Correspondence to Dr E Shmueh, MD Department of Oncology Ichilov Hospital Sorasky Medical Center 6 Weizman st Tel-Aviv 64239 Israel

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