Female Adnexal Tumor of Probable Wolffian Origin (FATWO): Case Report and Systematic Literature Review

Purpose: The paper aims to provide additional evidence and summarize the available evidence concerning the diagnosis, treatment, and prognosis of female adnexal tumors of probable Wolffian origin (FATWO). Methods: We presented a case of FATWO diagnosed and treated at our department. In addition, we performed systematic research of the literature (Scopus, PubMed/MEDLINE, ScienceDirect, and the Cochrane Library) including observational prospective and retrospective studies, case series, and case reports. We collected 165 cases of FATWO are available in the literature, including our case report. Results: We presented a case report of a 52-year-old asymptomatic woman with an incidental finding of an ovarian cyst. The final histological examination diagnosed it as an ovarian FATWO. Following surgical treatment alone, the patient continued to have negative follow-ups. The median age at diagnosis was 50 years old; the most frequent localization was the broad ligament (30,3%) followed by the ovary (26.1%), mesosalpinx (21.8%), and less common localizations. The most frequent symptom at the diagnosis was abdominal pain. Recurrent disease occurs in less than 20% of the cases with an overall survival of 59 months in patients with recurrent disease. Conclusions: FATWO is a rare cancer arising from mesonephric remnant most frequently from the broad ligament

How can we treat vulvar carcinoma in pregnancy? A systematic review of the literature

According to our systematic literature review (PRISMA guidelines), only 37 vulvar squamous cell carcinomas (VSCCs) were diagnosed during pregnancy (age range: 17\u201341 years). The tumor size range was 0.3\u201315 cm. The treatment was performed after (14/37, 38%), before (10/37, 27%), or before\u2010and\u2010after delivery (11/37, 30%). We found that 21/37 (57%) cases were stage I, 2 II (5%), 11 III (30%), and 3 IVB (8%). HPV\u2010related features (condylomas/warts; HPV infection; high\u2010grade squamous intraepithelial lesion) were reported in 11/37 (30%) cases. We also found that 9/37 (24%) patients had inflammatory conditions (lichen sclerosus/planus, psoriasis, chronic dermatitis). The time\u2010to\u2010recurrence/progression (12/37, 32%) ranged from 0 to 36 (mean 9) months. Eight women died of disease (22%) 2.5\u201348 months after diagnosis, 2 (5%) were alive with disease, and 23 (62%) were disease\u2010free at the end of follow\u2010up. Pregnant patients must be followed\u2010up. Even if they are small, newly arising vulvar lesions should be biopsied, especially in women with risk factors (HPV, dermatosis, etc.). The treatment of VSCCs diagnosed in late third trimester might be delayed until postpartum. Elective cesarean section may prevent vulvar wound dehiscence. In the few reported cases, pregnancy/fetal outcomes seemed to not be affected by invasive treatments during pregnancy. However, clinicians must be careful; larger cohorts should define the best treatment. Definite guidelines are lacking, so a multidisciplinary approach and discussion with patients are mandatory

Improved Risk Prediction in Human Papillomavirus-Associated Endocervical Adenocarcinoma Through Assessment of Binary Silva Pattern-based Classification: An International Multicenter Retrospective Observational Study Led by the International Society of Gynecological Pathologists (ISGyP)

Endocervical adenocarcinomas (EACs) are a group of malignant neoplasms associated with diverse pathogenesis, morphology, and clinical behavior. As a component of the International Society of Gynecological Pathologists International Endocervical Adenocarcinoma Project, a large international retrospective cohort of EACs was generated in an effort to study potential clinicopathological features with prognostic significance that may guide treatment in these patients. In this study, we endeavored to develop a robust human papillomavirus (HPV)-associated EAC prognostic model for surgically treated International Federation of Gynecology and Obstetrics (FIGO) stage IA2 to IB3 adenocarcinomas incorporating patient age, lymphovascular space invasion (LVSI) status, FIGO stage, and pattern of invasion according to the Silva system (traditionally a 3-tier system). Recently, a 2-tier/binary Silva pattern of invasion system has been proposed whereby adenocarcinomas are classified into low-risk (pattern A/pattern B without LVSI) and high-risk (pattern B with LVSI/pattern C) categories. Our cohort comprised 792 patients with HPV-associated EAC. Multivariate analysis showed that a binary Silva pattern of invasion classification was associated with recurrence-free and disease-specific survival (P < 0.05) whereas FIGO 2018 stage I substages were not. Evaluation of the current 3-tiered system showed that disease-specific survival for those patients with pattern B tumors did not significantly differ from that for those patients with pattern C tumors, in contrast to that for those patients with pattern A tumors. These findings underscore the need for prospective studies to further investigate the prognostic significance of stage I HPV-associated EAC substaging and the inclusion of the binary Silva pattern of invasion classification (which includes LVSI status) as a component of treatment recommendations

Management of sinonasal adenocarcinomas with anterior skull base extension

INTRODUCTION: Sinonasal adenocarcinomas (SNAC) are rare and heterogeneous. Management of SNAC follows a rather standardized and internationally accepted paradigm. Several refinements have been introduced during the last decade. METHODS: A narrative review of most updated literature on SNACs has been conducted. RESULTS: SNACs are classified as intestinal-type and non-intestinal-type, which are further categorized according to grade. Preoperative work-up should include magnetic resonance imaging (or contrast-enhanced computed tomography as a secondary or complementary choice) and biopsy under general anesthesia, or under local anesthesia in case of a history of exposure to wood and/or leather dust. Positron emission tomography, neck ultrasound, and fine-needle aspiration cytology are indicated in selected cases. Surgery represents the most common upfront modality of treatment and is usually accomplished via a transnasal endoscopic approach. Adjuvant radiation therapy is indicated for high-grade, locally advanced tumors and/or in case of margins involvement. Neoadjuvant chemotherapy with cisplatin, 5-fluorouracil and leucovorin may offer high response rates and long-term control in a subgroup of patients affected by intestinal-type adenocarcinoma, and in particular in those whose tumors harbor a functional p53 protein. Most of the bio- and immune-therapeutic potentials on SNACs still remain theoretical, and no clinical data are currently available. CONCLUSIONS: Management of SNAC consists of histological diagnosis, radiological staging, radical surgery, and adjuvant radiation therapy. Neoadjuvant chemotherapy can be indicated in selected cases. The role of biotherapy and immune therapy still needs to be elucidated

Prognosis and management of recurrent and/or metastatic head and neck adenoid cystic carcinoma

Adenoid cystic carcinoma (ACC) is a rare tumor, usually arising in the salivary gland, accounting for 1% of all head and neck cancers. ACC may have a long-term poor prognosis, as about 40% of radically treated patients will recur locoregionally and up to 60% will develop distant metastasis. Factors influencing risk of recurrence have been well studied, but few data exist about prognostic factors in Recurrent/Metastatic (RM) setting. Moreover, treatment of RM ACC is often a challenge for clinicians, in the context of a rare disease, which may have an indolent clinical behavior or less frequently a quicker growth and with a paucity of available clinical trials. This review critically analyzes pathological and molecular prognostic factors in RM ACC and make an overview on actual therapeutic choices and future direction of therapy. Recognized prognostic factors in RM ACC are the presence and site of distant metastasis (lung vs other), the presence of nodal metastasis and of extranodal extension, skull base recurrence, disease free interval, lymphovascular invasion, solid histotypes and grading of disease, and the presence of mutation of NOTCH1 family, PI3K, and TP53. Due to disappointing results with chemotherapy, new approaches are under study, also on the basis of biomolecular research. Ongoing clinical trials are evaluating treatment targeting MYB and NOTCH1 alterations, immunotherapy or combination of targeted treatments and immune checkpoint inhibitors