-
Pleura and Peritoneum Mar 2024There are limited treatment options and no consensus on the management of advanced rare ovarian malignancies. Rare ovarian malignancies can present with peritoneal...
OBJECTIVES
There are limited treatment options and no consensus on the management of advanced rare ovarian malignancies. Rare ovarian malignancies can present with peritoneal metastases (PM), featuring a similar presentation to more common ovarian subtypes. Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is an effective treatment for PM of non-gynecologic origin and, recently, epithelial ovarian cancer. We evaluated the feasibility of CRS/HIPEC in the management of PM from rare ovarian malignancies and report postoperative outcomes on these patients.
METHODS
A retrospective review of a single center, prospective database (1994-2021) was performed to identify patients with rare ovarian malignancies treated with CRS/HIPEC. Clavien-Dindo 90-day morbidity/mortality and Kaplan-Meier overall (OS) and progression-free survival (PFS) were analyzed.
RESULTS
Of 44 patients identified, 28 underwent CRS/HIPEC. Six were aborted due to extensive disease. Histologic subtypes included: clear cell (5/28, 17.9 %), endometrioid (5/28, 17.9 %), granulosa cell (3/28, 10.7 %), low-grade serous (6/28, 21.4 %), mesonephric (1/28, 3.6 %), mucinous (6/28, 21.4 %), and small cell (2/28, 7.1 %) carcinomas. Eight (28.6 %) patients had primary and 20 (71.4 %) had recurrent disease. Median peritoneal cancer index (PCI) was 21 (IQR: 6-29). Complete cytoreduction (<2.5 mm residual disease) was achieved in 27/28 (96.4 %). Grade III/IV complications occurred in 9/28 (32.1 %) with one (3.6 %) mortality. After a median follow-up of 65.8 months, 20 patients were alive. Five-year OS and PFS were 68.5 and 52.6 %, respectively.
CONCLUSIONS
In patients with PM from rare ovarian malignancies, CRS/HIPEC is feasible and has an acceptable safety profile. Longer follow-up and multicenter trials are needed.
PubMed: 38558871
DOI: 10.1515/pp-2023-0019 -
International Journal of Gynecological... Jul 2024Mesonephric-like adenocarcinoma (MLA) of the ovary is a recently recognized, rare malignancy with aggressive clinical behavior, and is thought to originate from...
Mesonephric-like adenocarcinoma (MLA) of the ovary is a recently recognized, rare malignancy with aggressive clinical behavior, and is thought to originate from Mullerian epithelium with mesonephric transdifferentiation. Emerging evidence suggests that MLA may be classified as an endometriosis-associated neoplasm. The presence of a sarcomatous component within MLA is extremely rare, with common differential diagnoses including the spindle cell component of MLA, carcinosarcoma, as well as mixed Mullerian adenocarcinoma and adenosarcoma. Herein, we report a 58-year-old Chinese woman with bilateral ovarian solid-cystic masses. The left ovarian mass comprised a biphasic tumor with a predominantly high-grade sarcomatous component displaying heterologous mesenchymal differentiation, including liposarcoma, rhabdomyosarcoma and chondrosarcoma-like areas, with a null-type p53 expression. The epithelial component ranged from a bland appearance in areas diagnostic of adenosarcoma to a clearly invasive carcinoma, both with mesonephric-like phenotype, being negative for estrogen receptor, progesterone receptor, and Wilms' tumor 1, variably positive for paired box gene 8, GATA binding protein 3, and thyroid transcription factor 1, with a wild-type p53 expression. The differing p53 expression between the epithelial and sarcomatous elements mitigated against a diagnosis of carcinosarcoma. The right ovarian mass showed endometriosis with focal direct evidence of the development of malignancy within a benign endometriotic cyst, exhibiting the identical immunoprofile of MLA but originating as another malignancy. To the best of our knowledge, this case represents the first reported case of synchronous bilateral ovarian MLAs with separate origins, from high-grade Mullerian adenosarcoma and endometriosis respectively, which broadens the morphologic spectrum of MLA and provides further evidence supporting the Mullerian origin theory.
Topics: Humans; Female; Endometriosis; Middle Aged; Adenosarcoma; Ovarian Neoplasms; Adenocarcinoma; Ovary; Diagnosis, Differential; Mixed Tumor, Mullerian
PubMed: 37922943
DOI: 10.1097/PGP.0000000000000997 -
Journal of UOEH 2024Adenocarcinoma, HPV-independent, mesonephric type (hereafter referred to as "mesonephric carcinoma") arising from the cervix is rare, its treatment has not been...
Adenocarcinoma, HPV-independent, mesonephric type (hereafter referred to as "mesonephric carcinoma") arising from the cervix is rare, its treatment has not been established, and its sensitivity to chemotherapy has not been fully investigated. Here we report on a 30-year-old female patient who presented at our hospital with a chief complaint of abnormal genital bleeding. We suspected cervical cancer. Based on examination, biopsy, and imaging, she was diagnosed with stage IIA2 adenocarcinoma of the cervix and was scheduled for surgery. Because she had a SARS-COV-2 infection, she was given two courses of paclitaxel-carboplatin (TC) therapy, based on the then-current surgical risk assessment after SARS-COV-2 infection, with a waiting period of at least 8 weeks. The patient was deemed to have a partial response and was treated with paclitaxel and carboplatin, after which she was deemed to have a partial response and underwent total hysterectomy. A diagnosis of stage IIA2 mesonephric carcinoma, ypT1b2N0M0, was made after histopathologic examination of an excised specimen. The patient was treated with 4 additional courses of TC therapy after surgery, and has had no recurrence in 13 months. We report a first case of response to neoadjuvant chemotherapy with TC regimen in a patient with mesonephric carcinoma of the cervix.
Topics: Female; Humans; Adult; Carboplatin; Neoadjuvant Therapy; Papillomavirus Infections; Adenocarcinoma; Uterine Cervical Neoplasms; Mesonephroma; Paclitaxel; COVID-19
PubMed: 38479874
DOI: 10.7888/juoeh.46.45 -
Histopathology May 2024
The first evidence of mismatch repair deficiency in mesonephric-like adenocarcinoma of the endometrium: clinicopathological and molecular features of a case emphasising a possible endometrioid carcinogenesis.
Topics: Female; Humans; Adenocarcinoma; Endometrium; Brain Neoplasms; Neoplastic Syndromes, Hereditary; Carcinoma, Endometrioid; Endometrial Neoplasms; Colorectal Neoplasms
PubMed: 38173293
DOI: 10.1111/his.15136 -
Asian Journal of Surgery Feb 2024
Topics: Female; Humans; Brenner Tumor; Ovarian Neoplasms; Pelvis; Cysts
PubMed: 37981498
DOI: 10.1016/j.asjsur.2023.11.033 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Feb 2024
Topics: Female; Humans; Endodermal Sinus Tumor; Adenocarcinoma; Uterus; Biomarkers, Tumor
PubMed: 38281794
DOI: 10.3760/cma.j.cn112151-20231016-00272 -
Zhonghua Bing Li Xue Za Zhi = Chinese... Feb 2024
Topics: Female; Humans; Urinary Bladder; Endometriosis; Adenocarcinoma; Urinary Bladder Neoplasms
PubMed: 38281795
DOI: 10.3760/cma.j.cn112151-20231007-00232