Conjunctival melanoma (CM) is an exceptionally rare tumor, with a propensity for local and distant recurrence, with the lungs, skin, liver, and brain being the most common sites of metastasis. Recent progress in systemic treatments, with checkpoint inhibitors and targeted therapies blocking BRAF and MEK, has redefined the standard of care of advanced unresectable and metastatic melanoma. Although most trials did not include patients with conjunctival melanoma, its close molecular and genetic relationship to cutaneous melanoma might suggest a similar response to these novel agents. The authors describe two uncommon cases of metastatic conjunctival melanomas with distinct genetic profiles and, as such, submitted to different systemic treatments.

Neuroendocrine tumors (NETs) are rare, representing 0.5% of all newly diagnosed malignancies. Rectal and anal canal (AC) NETs account for less than 1% of all rectal and AC cancers. Review our institutional experience on NET of the rectum and AC, with emphasis on demographic, histological and treatment features and oncologic outcomes. The study group was identified from the Portuguese Regional South Oncological Registry. From 2000 to 2014, 22 patients with rectal or AC NETs were treated at our institution. Medical records were retrospectively reviewed. There were 12 males (54.5%) and 10 females (45.5%) and the median age at diagnosis was 59.5 years. The majority had rectal NET (81.8%). All 4 patients with AC NETs had neuroendocrine carcinoid (NEC) tumors. Of the patients with rectal NETs, 3 had NEC and 15 had NET, mainly G1. Different approaches to treatment were made according to histological and staging features. After an average follow-up of 39.1 months, 16 patients were alive and only one with evidence of disease. The median time to progression was 12.4 months and the liver was the most frequent site of metastasis. The European and North American Neuroendocrine Societies offer guidelines for the treatment of rectal NETs. However, for AC NETs there are only small series and not prospective studies due to their rarity, hence the importance to report institutional experience. Our practice demonstrated that primary excisional treatment, regardless the histology, provides a favorable prognosis and long survival.

Introduction: intestinal-type sinonasal adenocarcinoma (ITAC) is a rare epithelium tumor of the nasal cavities and paranasal sinuses. Exposure to wood and leather dusts is a strong etiological factor related to its development. Prolonged cork exposure has rarely been associated. Materials and Methods: thirty-seven-year (1981-2018) retrospective cohort analysis of all consecutive patients with sinonasal cancer (SNC) followed at our institution. Medical records were reviewed to determine patient demographics, occupational/environmental exposure, location and extent of the tumor, stage, histopathology findings, treatment strategies, and oncologic outcomes. Survival analysis was done using Kaplan-Meier method. Results: we evaluated 379 patients with SNC, including 39 (10.3%) ITAC. Patient median age was 73 years (range 49-87), 56% male and 69% with identified professional occupational exposure (54% for cork; 69.2% considering only those for which an agent has been identified). Seventy-two percent had locally advanced disease (stage III or IVA-B). The initial treatment was surgery in 77%, and 54% received adjuvant radiotherapy. The median time to progression, progression-free survival, and overall-survival was 2.36 years (95% CI 1.54-8.70), 1.96 years (95% CI 1.43-3.74), and 3.51 years (95% CI 2.33-10.02), respectively. Conclusion: ITAC is an uncommon malignancy that grows silently, which contributes to delayed diagnosis, advanced stage and low survival rates. In our cohort, we observed a high prevalence of cork occupational exposure. This finding may lead to the implementation of protection measures and suggest a potential link to be further studied.

Introduction Ovarian cancer is a leading cause of death among women with gynecologic malignancies. The relapse rate is high after platinum-based therapy, with the effectiveness of subsequent treatment lines decreasing over time. Recent data suggest the benefit of maintenance therapy with niraparib in platinum-sensitive recurrent disease. Case Presentations We report a case series of five women with advanced ovarian cancer and BRCAness phenotype who responded favorably, and in some cases with long-term response, to maintenance therapy with niraparib. Toxicities were as expected and generally manageable. Two patients developed grade 2/3 hematological toxicity, which resolved with treatment suspension and subsequent dose reductions, and one patient reported a rare skin toxicity while responding to full-dose niraparib treatment, which was controlled with photoprotection and sunscreen. Discussion and Conclusions This case series highlights the role of PARP1/2 inhibitors as a new standard of care as maintenance therapy for recurrent platinum-sensitive high-grade ovarian cancer, irrespective of BRCA status.

Introduction. Primitive malignant heart tumours are rare, specific cases. The presence of cardiac metastases, often in the pericardium, besides indicating disseminated oncological disease, represents a diagnostic challenge since they tend to be asymptomatic. Malignant cutaneous melanoma (MCM) is the neoplasm that most often affects the heart. Patients and Methods. The authors describe a case report of a 59-year-old female patient with a history of non-insulin-treated diabetes mellitus, arterial hypertension, dyslipidemia, and remitting cutaneous malignant melanoma who underwent skin excision, lymphadenectomy, and adjuvant chemotherapy in 1996. In April 2014, she resorted to emergency service due to epigastric pain and progressive tiredness. Due to the persistence of the complaints, abdominal ultrasound was performed, which showed a large pericardial effusion, corroborated later by teleradiography and echocardiography. The patient underwent pericardiocentesis, which isolated neoplastic cells. A computed tomography study of the chest, abdomen, and pelvis revealed bilateral and pericardial pleural effusion, as well as alterations suggestive of pericardial and pulmonary metastasis. Later, fine-needle aspiration puncture of the left posterior cervical nodule confirmed histologically malignant melanoma metastasis. Discussion. Given the natural history of melanoma that when metastasized has an overall survival of 15–20% for 5 years, its metastatic spread may occur several years after its surgical excision. Thus, patients with a history of melanoma and heart failure who develop new cardiac symptoms of unknown aetiology should undergo imaging studies such as echocardiography, computed tomography, and magnetic resonance imaging.