RESUMO
PURPOSE: Ovarian cancer is the most common deadly cancer of gynecologic origin. Patients often are diagnosed at advanced stage with peritoneal metastasis. There are many rare histologies of ovarian cancer; some have outcomes worse than serous ovarian cancer. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) can be considered for patients with recurrence. This study was designed to assess the impact of CRS and HIPEC on survival of patient with peritoneal metastasis from rare ovarian malignancy. METHODS: A prospective, multicentric, international database was retrospectively searched to identify all patients with rare ovarian tumor (mucinous, clear cells, endometrioid, small cell hypercalcemic, and other) and peritoneal metastasis who underwent CRS and HIPEC through the Peritoneal Surface Oncology Group International (PSOGI) and BIG-RENAPE working group. The postoperative complications, long-term results, and principal prognostic factors were analyzed. RESULTS: The analysis included 210 patients with a median follow-up of 43.5 months. Median overall survival (OS) was 69.3 months, and the 5-year OS was 57.7%. For mucinous tumors, median OS and DFS were not reached at 5 years. For granulosa tumors, median overall survival was not reached at 5 years, and median DFS was 34.6 months. Teratoma or germinal tumor showed median overall survival and DFS that were not reached at 5 years. Differences in OS were not statistically significant between histologies (p = 0.383), whereas differences in DFS were (p < 0.001). CONCLUSIONS: CRS and HIPEC may increases long-term survival in selected patients with peritoneal metastasis from rare ovarian tumors especially in mucinous, granulosa, or teratoma histological subtypes.
Assuntos
Carcinoma Endometrioide/terapia , Procedimentos Cirúrgicos de Citorredução , Tumor de Células da Granulosa/terapia , Hipertermia Induzida , Neoplasias Císticas, Mucinosas e Serosas/terapia , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Ovarianas/patologia , Neoplasias Peritoneais/terapia , Teratoma/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Endometrioide/secundário , Procedimentos Cirúrgicos de Citorredução/efeitos adversos , Intervalo Livre de Doença , Feminino , Seguimentos , Tumor de Células da Granulosa/secundário , Humanos , Metástase Linfática , Pessoa de Meia-Idade , Neoplasias Císticas, Mucinosas e Serosas/secundário , Neoplasias Embrionárias de Células Germinativas/secundário , Neoplasias Peritoneais/secundário , Doenças Raras/patologia , Doenças Raras/terapia , Estudos Retrospectivos , Taxa de Sobrevida , Teratoma/secundário , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Pseudomyxoma peritonei (PMP) usually originates from appendiceal neoplasms and, less commonly, from extra-appendiceal lesions. To date, the clinical and therapeutic implications of extra-appendiceal origin are largely unknown. METHODS: A prospective database of 225 PMP patients uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) was reviewed to identify cases with extra-appendiceal primaries. Histologically, negative appendix defined extra-appendiceal origin. Clinical, pathological, and immunohistochemical features (cytokeratin [CK]-20, CK-7, CDX-2, MUC-2, MUC-5A) were correlated with the site of origin. PMP was categorized into low or high grade, according to the 2010 World Health Organization (WHO) classification. The main independent variable for survival analysis was appendiceal versus extra-appendiceal primary. RESULTS: In 19 patients (8.4 %), PMP origin was the ovary (n = 9), uterine cervix (n = 1), mature cystic teratomas (n = 4), and unknown (n = 5). Appendiceal and extra-appendiceal PMP groups were comparable for all characteristics, except for a prevalence of females in the latter. Median follow-up was 64.1 months (95 % confidence interval [CI] 53.9-80.1), and 10-year overall survival was 63.4 % (median 148.2 months; 95 % CI 131.2-165.2) for appendiceal PMP, and 62.0 % (median not reached) for extra-appendiceal PMP. The difference was not significant at univariate ( p = 0.297) and multivariate analysis (hazard ratio 1.51, 95 % CI 0.78-3.14; p = 0.278). High-grade peritoneal histology (p = 0.007), prior systemic chemotherapy (p = 0.003), more than four visceral resections (p = 0.011), and incomplete cytoreduction (p = 0.021) independently correlated with poorer survival. CONCLUSIONS: Clinical-pathological features of PMP, and outcome after CRS/HIPEC, did not differ according to the primary site, thus suggesting that PMP is a relatively homogeneous disease that can be produced by a range of histopathologic entities. Extra-appendiceal origin does not contraindicate CRS/HIPEC.
Assuntos
Neoplasias do Apêndice/patologia , Neoplasias Primárias Desconhecidas/patologia , Neoplasias Ovarianas/patologia , Neoplasias Peritoneais/terapia , Pseudomixoma Peritoneal/terapia , Teratoma/secundário , Neoplasias do Colo do Útero/patologia , Adulto , Idoso , Antineoplásicos/administração & dosagem , Fator de Transcrição CDX2/metabolismo , Procedimentos Cirúrgicos de Citorredução , Feminino , Humanos , Hipertermia Induzida , Queratina-20/metabolismo , Queratina-7/metabolismo , Masculino , Pessoa de Meia-Idade , Mucina-5AC/metabolismo , Mucina-2/metabolismo , Gradação de Tumores , Neoplasia Residual , Neoplasias Peritoneais/secundário , Pseudomixoma Peritoneal/patologia , Reoperação , Estudos Retrospectivos , Fatores de Risco , Taxa de SobrevidaRESUMO
A 29-year-old man presented with a right testicular mass. Serum tumor markers were within normal limits. When compared to a previous computed tomography (CT) scan, a new 4 cm presacral mass was present. He underwent radical right inguinal orchiectomy that demonstrated a mature teratoma and seminomatous components. The patient received four cycles of chemotherapy. Over the course of chemotherapy, the mass grew in size and therefore he underwent retroperitoneal lymph node dissection. Pathology confirmed it to be a teratoma with negative retroperitoneal lymph nodes. The unusual presentation of an isolated metastasis to the presacral region raises the question of altered lymphatic drainage.