Invasive and noninvasive implants in ovarian serous tumors of low malignant potential

Ovarian serous tumors of low malignant potential ("borderline" serous tumors) are classified according to the histologic features of the primary ovarian tumor, without regard to any coexisting extraovarian disease. The peritoneal implants display a range of histologic appearances, ranging from benign glands (endosalpingiosis), to noninvasive papillary glandular proliferations resembling the ovarian neoplasms, to irregular glands associated with a desmoplastic stroma and having features of invasive disease. This review of 16 patients with histologically documented extraovarian tumor implants seen at Indiana University Medical Center, Indianapolis, and 13 patients whose tumor implants have been previously described in the literature indicates that the clinical stage of disease has much greater prognostic significance than does the implant histologic features. There is a tendency for patients with more advanced disease to have invasive implants. However, within a given clinical stage, disease progression or recurrence was not influenced by the presence or absence of invasive histologic characteristics in the tumor implants.     

Extraovarian peritoneal serous papillary carcinoma showing complete remission after treatment with cisplatin

Extraovarian peritoneal serous papillary carcinoma is a rare disease in women that arises from extraovarian peritoneum with müllerian potential. It shows histopathological findings identical to those of ovarian serous carcinoma. At diagnosis, this disease usually exhibits peritoneal carcinomatosis, but evidence of a primary site is lacking. We experienced a case of extraovarian peritoneal serous papillary carcinoma in a 72-year-old woman who complained of abdominal distension and soon after developed bowel obstruction. Although we failed to perform any effective surgical procedure at laparotomy, because the cancer was extremely advanced, treatment with cisplatin resulted in rapid reduction of the tumor, and, finally, in its complete disappearance. Cisplatin was administered only three times, and the total dose was 300 mg. The patient is still alive and clinically free of recurrence 32 months after her first visit to our hospital. In this report we describe her clinical course, and discuss the diagnosis and treatment of this disease, and the usefulness of tumor markers for monitoring it. Received: February 22, 2000 / Accepted: May 29, 2000     

Adenocarcinoma papilar seroso primario del peritoneo

A clinical case of primary peritoneal serous adenocarcinoma is presented. A woman 66 years old with abdominal pain and ascitis. Clinical, radiologic and cytology data suggested a peritoneal carcinomatosis of an unknown primary adenocarcinoma. Laparotomy and definitive histopathological findings show a müllerian primary peritoneal serous adenocarcinoma with normal ovarian stromal tissue. A similitude with stage III ovarian adenocarcinoma has led to the Gynecologic Oncology group to make a criteria for these neoplasia: normal ovarian size; non ovarian stromal tissue affectation; more extraovarian disease; histopathology of a papillary serous carcinoma. Nevertheless the correct treatment for these type of tumor is the same as that of the ovarian adenocarcinoma.     

11例正常大小卵巢癌综合征的临床及病理分析

目的探讨正常大小卵巢癌综合征的临床特点、诊断标准、有效的治疗方法及其预后。方法回顾性分析１９８４～１９９３年在我院治疗及病理复核证实的１１例正常大小卵巢癌综合征,其中７例为性腺外苗勒氏管肿瘤,也称卵巢外腹膜浆液性乳头状腺癌（ＥＰＳＰＣ）,２例卵巢浆液性腺癌,１例恶性间皮瘤,１例原发灶不明的转移性腺癌。上述无论何种类型,均宜最大限度切除肿瘤,术后残存肿瘤＜２ｃｍ,并辅以有效多程综合化疗。结果１例恶性间皮瘤存活９０个月,随访至１９９７年６月仍健在。１例ＥＰＳＰＣ已存活８７个月,随访至１９９７年１１月仍健在。９例已死亡患者平均生存１７．８个月,比文献报道存活时间长。结论对此综合征患者必须高度重视鉴别诊断,首选手术治疗,尽量切除癌灶（残存肿瘤＜２ｃｍ）,术后辅以有效化疗或放疗,以获得最佳生存和预后。     

Primary papillary serous carcinoma of the peritoneum in a man

Background:Primary papillary serous carcinoma of the peritoneum is a well-known entity in women. The tumour is derived from the extraovarian 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 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.     

A case of extraovarian primary peritoneal carcinoma successfully treated with weekly paclitaxel

We report the use of paclitaxel in the successful treatment of a 74-year-old patient with extraovarian primary peritoneal carcinoma and acute renal failure caused by intraperitoneal cisplatin. The histological diagnosis was papillary serous adenocarcinoma. Paclitaxel was given intravenously at 70 mg/m2 in a 1-hour infusion weekly for 6 consecutive weeks followed by 2-weeks rest (one cycle). Toxicities including neutropenia and neurotoxicity were mild. She showed a clinical response after three cycles, and then underwent secondary cytoreductive surgery after which she received one additional cycle of therapy. She enjoyed a favorable quality of life without evidence of disease for 16 months after the completion of this therapy. Weekly 1-hour paclitaxel (70 mg/m2) was well tolerated, yet was effective for extraovarian primary peritoneal carcinoma.     

Survival among women with borderline ovarian tumors and ovarian carcinoma: a population-based analysis

BACKGROUND: Serous and mucinous ovarian tumors of low malignant potential (LMP-S and LMP-M, respectively) are noninvasive tumors that portend excellent survival when confined to the ovary. Comparison of the survival for women with LMP tumors staged as distant with women who have carcinoma may have important implications for diagnostic terminology and clinical management. METHODS: The authors compared relative survival rates among patients diagnosed with ovarian tumors during the period 1988-1999 (with follow-up through 2000) by histologic type, disease stage, tumor grade (for carcinomas), and patient age, using data from the Surveillance, Epidemiology, and End Results Program. RESULTS: The overall relative survival rate at 10 years (+/- 1.96 standard errors) was 96.9% +/- 2.3% for women with LMP-S tumors, 30.4% +/- 1.7% for women with serous carcinoma (CA-S); 94.0% +/- 3.1% for women with LMP-M tumors, and 64.7% +/- 3.4% for women with mucinous carcinoma (CA-M). The survival rate at 10 years for women with distant-stage LMP-S tumors was 89.9% +/- 5.3%, compared with 96.1% +/- 8.6% for women with well differentiated, localized CA-S. The survival rate for women with distant-stage LMP-M tumors at 5 years was 85.5% +/- 9.0%, compared with 95.5% +/- 3.4% for women with well differentiated, localized CA-M (data for 10 years were limited). Mucinous ovarian neoplasms were associated with an excess of second malignancies of the digestive tract. CONCLUSIONS: Relative survival among women with distant-stage LMP tumors was not 100% and resembled the survival of women who had carcinoma exhibiting favorable prognostic features (localized stage). Future studies of women with high-stage LMP tumors are required to clarify the pathogenesis of extraovarian lesions and their implications for management and prognosis.     

Serous papillary adenocarcinoma of the tunica vaginalis of the testis with metastasis

Testicular or paratesticular neoplasms that resemble the common epithelial type of ovarian tumor are quite rare. The authors report the case of a 29-year-old man with a metastatic serous papillary adenocarcinoma arising from the tunica vaginalis. To the authors' knowledge, this is the first reported case of a serous carcinoma of the tunica vaginalis behaving in a malignant fashion. The fact that clinically apparent metastatic disease occurred 4 years after initial presentation suggests that development of metastases is a late event. Unfortunately, the tumor has been refractory to therapy with chemotherapeutic agents with activity against ovarian malignancies.     

卵巢外腹膜乳头状癌：（附6例报告）

卵巢外腹膜乳头状癌少见,发病缓慢而隐袭,常占满全腹或腹腔的一部分,呈乳头或结节状,播散性绒毛或粟粒状,可为大小不等的实性肿块,无包膜,广泛粘连或融合成团,肿物似在腹腔内广泛播散,很少累及腹腔外,通常卵巢正常或浅表受累,病理光镜下结构一如卵巢浆液性乳头状癌,常见砂粒体,故常误诊为卵巢癌腹膜转移,本文报告6例,年龄23～72岁,平均43岁,较文献中偏低。治疗以手术尽量切除,化疗以卵巢癌方案,6例中存活30个月2例,2例带瘤生存分别达12、14个月,另2例术后12、20个月死亡。     

Density distribution and antigenic characterization of cell subpopulations isolated from cystic fluids of ovarian serous neoplasms

The immunological reactivity of serous ovarian tumor cells was evaluated, taking into account their density and morphological features. Discontinuous density gradient centrifugation was applied to fractionate cell subpopulations from cystic fluids of patients with ovarian serous carcinomas, cystadenomas and benign serous cysts. For phenotypic characterization of tumor cell subpopulations the immune sera against perchloric acid extracts of ovarian serous (anti-PCA-CaOs) and mucinous (anti-PCA-CaOm) carcinomas were used. The expression of carcinoembryonic antigen (CEA) and nonspecific cross-reacting antigen (NCA) on tumor cell fractions was also checked. Some relationship between immunological reactivity, cell morphology and cell density was found; however, individual patient-to-patient variations in cellular composition and antigenic expression were also observed. The presence of ovarian serous carcinoma-associated antigen (CaOs-Ag) was related mainly to frankly malignant cells. Neither CEA nor NCA were constant and characteristic markers for serous ovarian neoplasms. These neoplasms were unreactive with anti-PCA-CaOm serum. Our results indicate the possibility of correlation between morphological features, density distribution and immunological phenotypes of ovarian tumor cell populations.     

Primary papillary serous carcinoma of the peritoneum: Four cases and review of computed tomography findings

Primary papillary serous carcinoma of the peritoneum is an uncommon primary malignancy of the peritoneum and is histologically indistinguishable from papillary serous carcinoma of the ovary. The diagnosis of primary peritoneal papillary serous carcinoma should be considered in the presence of peritoneal and omental masses in the absence of an ovarian mass. Although it has been extensively documented in the pathological and gynaecological oncology literature, the CT appearance of primary papillary serous carcinoma of the peritoneum has been reported in only 51 cases in five reports. We present four patients with CT findings of pathologically proven primary papillary serous carcinoma of the peritoneum. There were a total of 23 patients with a histopathologically proven diagnosis of primary papillary serous carcinoma of the peritoneum between 1980 and 2002 with CT imaging. However, only four of the 23 patients’ CT films were retrieved for retrospective evaluation. The rest of the films were not available as either patients had misplaced the films or patients were deceased.     

Pathology of ovarian carcinoma

Serous carcinoma is the most common type of ovarian cancer and usually is associated with peritoneal metastases and poor survival except for meticulously staged patients with tumors confined to the ovaries. Endometrioid and clear cell carcinomas account for most nonserous carcinomas and more often present with low-stage disease; survival for the various cell types is similar when stratified by stage. Borderline ovarian tumors can be subdivided into benign and malignant neoplasms, and in the view of some experts, this renders the borderline category obsolete. Women with typical serous borderline tumors (atypical proliferative serous tumors) constitute most of these patients and have virtually 100% survival, unless invasive peritoneal implants are present. Micropapillary serous carcinomas (a less common variant, also called serous borderline tumor with a micropapillary pattern) and tumors with invasive implants behave similar to low-grade invasive carcinomas.     

卵巢外腹膜浆液性乳头状癌诊疗进展

卵巢外腹膜浆液性乳头状癌是一种原发于腹膜的恶性肿瘤,来源于第二苗勒系统,其临床表现及组织学特征与卵巢浆液性乳头状癌相似,术前误诊率高,确诊主要依靠组织病理学检查及免疫组化指标。治疗原则以手术为主,术后辅以铂类为基础的联合化疗。分化程度低、侵袭性强、预后较差、病死率高。近几年对该病的认识有所提高,相关报道不断增多,现对其临床研究进展进行分析。     

Clinicopathological aspects of pancreatic cancer

Using the nationwide database of the Japan Pancreas Society (JPS), the clinicopathological features of 23,284 cases (1981-2000) and 2,298 cases (2001-2002) with pancreatic neoplasms were compared. Intraductal papillary mucinous neoplasms and mucinous cystic neoplasms were increased in the registry. More detailed histological repertoires of endocrine tumors, intraductal tubular tumors and solid-pseudopapillary tumors were registered in the last two years. The numbers of serous cystadenocarcinomas and carcinomas in situ were decreased. The proportion of less differentiated adenocarcinoma was increased in the more advanced stages of the disease. In Stage IVa, the survival of the patients with papillary adenocarcinoma was not statistically different from that of patients with well or moderate tubular adenocarcinoma, though the difference was significant in earlier stages. The survival of the patients with poorly differentiated adenocarcinoma, adenosquamous carcinoma and undifferentiated carcinoma was miserable. Histological confirmation is critically important to prospect the outcome and to determine the treatment modality. Integration of the nationwide registry and pathological information will give new insights for the treatment of pancreatic cancer.     

Peritoneal papillary serous carcinoma with long-term survival

Three cases of peritoneal papillary serous carcinoma with a complete response to chemotherapy and long-term disease free survival are reported. All three patients presented with multifocal or diffuse peritoneal involvement. Microscopic ovarian serosal surface involvement was demonstrated in two cases. The ovaries were uninvolved in one case. All patients were treated with combination chemotherapy consisting of Adriamycin (doxorubicin) and cisplatin, with or without cytoxan. All three patients survived for 5 years or longer with no clinical evidence of persistent or recurrent tumor.     

Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases.

Fifty-eight immature ovarian teratomas were studied. Neoplams with other germ cell elements (endodermal sinus tumor, choriocarcinoma, and dysgerminoma) were excluded so that the clinical and pathologic features of "pure" immature teratomas could be defined and correlated with the prognosis. The primary tumors and their metastatic growths were graded from 0 to 3. Forty were stage I; nine, stage II; and nine, stage III. The size and stage of teratomas were related to survival, but it was the grade of the primary tumor that best determined the likelihood of extraovarian spread, and it was the grade of the metastases that related best to the subsequent course. Actuarial survival was 63% at 5 years and also at 10 years. Regardless of the grade of the primary tumor, only one of six with grade 0 metastases progressed, and that neoplasms may not have been adequately sampled. Two of five neoplasms having grade 1 metastases did not progress, and two of six patients with grade 2 metastatic growths were living after relatively long intervals. All seven patients with grade 3 metastases died with tumor, none surviving more than 2.1 years. Survival of patients with grade 1, 2, and 3 neoplasms was 81, 60, and 30% respectively. The importance of adequate sampling of primary tumor and metastases for estimating prognosis and determining therapy is stressed.     

Involvement of chromosome 6 in endometrial cancer.

Cytogenetic investigation was performed on direct preparations of 15 endometrial cancers showing different histotypes. Clonal abnormalities were found in 11 out of 13 analysable cases. The modal chromosome number was near diploid in all cases. The abnormal karyotypes contained relatively simple numerical or structural aberrations in the majority of tumours. In contrast, two neoplasms with serous papillary and mixed mullerian morphological features shared multiple complex changes as well as cytogenetic evidence of intratumoral heterogeneity. The most frequent chromosome abnormality in our series of endometrial neoplasms was 6q deletion, which was detected in serous papillary, endometrioid and mixed mullerian tumours. The loss of the 6q region, which is also frequently involved in ovarian carcinoma, suggests a relationship between endometrial and ovarian cancers based on a common histogenesis.     

Prognostic factors of secondary ovarian carcinoma

OBJECTIVE: Although the ovaries are common sites of metastases from a variety of primary neoplasms excluding carcinomas of the genital tract, there were few reports concerning survival and prognostic factors. The objective of this study was to assess the clinical factors affecting survival. METHODS: Fifty-three secondary ovarian carcinomas excluding metastases from genital tract carcinoma were registered by the Tokai Ovarian Tumor Study Group from 1989 to 1999. FIGO staging was set without considering the pathologic findings of the lymph nodes and the primary tumors. RESULTS: Twenty-four patients were stage I, 11 were stage II, 16 were stage III, and 2 were stage IV. There were significant differences in the survival curves between the early stages (I and II) and advanced stages (III and IV). The 5-year survival rate for patients without residual tumors was 39.9%, while all patients with residual tumors after surgery died within 33 months. The most frequent primary tumor was large intestinal carcinoma, and the second was gastric carcinoma. The 5-year survival rate for patients with large intestinal primary tumors was significantly better than that with stomach tumors. Multivariate analysis demonstrated that tumor stage and primary tumor sites were significant prognostic factors. CONCLUSION: FIGO staging without considering lymph node involvement and the primary tumor was a significant prognostic factor, and prognostic factors for primary ovarian carcinomas can be applied to secondary ovarian carcinomas. Furthermore, the primary tumor site was also an important prognostic factor for survival.     

Prognosis of stage III or IV primary peritoneal serous papillary carcinoma.

AIMS: To study the prognosis of patients with stage IIIC/IV primary peritoneal serous papillary carcinoma (PSPC) (study group) compared with that of patients with epithelial ovarian carcinoma (EOC) (control group). METHODS: A retrospective case-control study including a study group of 37 patients who were matched with a control group of 37 patients. Patients were matched for the histologic subtype (serous tumor), tumor stage, tumor grade, residual disease at the end of debulking surgery (initial or interval) and age (+/-5 years). RESULTS: Debulking surgery was performed initially or at interval surgery in respectively, 10 and 27 patients in the study group and 17 and 20 in the control group. All patients were treated with platinum-based chemotherapy (combined with paclitaxel in 33) in both groups. The overall survival rate at 3 years in the study and control groups was, respectively, 60% versus 55% (NS). However, event-free survival rates at 3 years (CI 95%) were statistically different (respectively, 29% in the study group versus 16% in the control group: p=0.008). CONCLUSIONS: Peritoneal disease is more bulky in patients with PSPC. Neoadjuvant chemotherapy is more often required to achieve optimal debulking surgery in PSPC. Overall survival of patients with PSPC is similar to that of their EOC counterparts. Thus, the management of PSPC should not be different from that of advanced stage EOC.