Ovarian Borderline Tumor and Fertility-Sparing Surgery

Ovarian borderline tumors (OBTs) are frequently diagnosed in women of reproductive age. There is no consensus about their management, and it sometimes represents a dilemma aboutwhat should be done: fertility sparing surgery or a hysterectomy with salpingo-oophorectomy? Case: A 32-year-old nulligravida, diagnosed with a right ovarian borderline tumor is presented. She underwent pelvic washings, right salpingo-oophorectomy, appendectomy, and omental and peritoneal biopsies (laparotomic approach). Macroscopically, the left ovary was normal and subsequent exploration for staging was also normal, including the lymph nodes. Intraoperatively, frozen section examination was unclear, suggesting an OBT. Results: The final histopathologic diagnosis was ovarian borderline tumor, stage IIC (International Federation of Gynecology and Obstetrics [FIGO] staging). The patient expressed a desire to preserve her fertility. Thirty-six months postsurgery, she became pregnant spontaneously and delivered a healthy newborn at term. Conclusions: Conservative surgery can be performed in young patients treated for an OBT, provided they are closely followed up and that this surgery is performed after careful consideration and informed consent. It is, however, controversial with respect to performing hysterectomy and salpingo-oopherectomy upon the patient’s completion of childbearing.

A Rare Abdomino-Pelvic Tumor: Paraganglioma

Paragangliomas are rare tumors, with a reported incidence of 2–8 per million. They are chromaffin cell tumors that develop from the neural crest cells and may be divided in tumors derived from the parasympathetic or sympathetic ganglia. We report a case a of a 32-year-old nulliparous woman, referred to our Infertility Clinic. Abdomino-pelvic ultrasound identified a large abdominopelvic tumor, without ovarian origin (both ovaries were identified and had normal morphology). Magnetic Resonance Imaging suggested a right adnexal multicystic, vascularized mass close to iliac vessels and questioning an ovarian origin. At exploratory laparotomy, a 10 cm encapsulated and vascularized mass was found beginning just below right renal artery and extending to the level of the broad ligament. This mass was totally excised and histopathology was consistent with Paraganglioma.

Diagnosis of Eosinophilic Esophagitis in an Infant Undergoing Milk Oral Immunotherapy - A Case Report

Although the standard of care for cow’s milk (CM) allergy is strict food avoidance, oral immunotherapy (OIT) is being widely investigated as an alternative management option in certain cases. Immediate adverse reactions to OIT have been described, but its long-term effects are much less often reported. We present the case of a girl diagnosed with IgE-mediated CM allergy that was proposed for our CM OIT protocol at the age of 3 years. The first sessions (dose escalation up to 5 ml) were well tolerated, however eight hours after her daily morning dose of 5ml CM the child developed late episodes of vomiting. No other symptoms, particularly immediately after CM ingestion, were reported. These episodes became progressively worse and on the third day she presented mild dehydration and blood eosinophilia. After OIT interruption, a progressive clinical improvement was observed. An esophageal endoscopy was performed, showing signs of eosinophilic esophagitis (EoE) with peak 20 eosinophils/hpf. After treatment with topical swallowed fluticasone (500 mcg bid) and a CM-free diet for 4 months, the child was asymptomatic and endoscopy and biopsy findings were normal. The long-term effects of milk OIT are still in part unknown. We hypothesize that eosinophilic esophagitis may have been a consequence of OIT in this case. The findings seem to indicate that food allergy may play a role in the pathogenesis of esophageal eosinophilia and stress the importance of a well programmed long-term follow-up of patients that have undergone milk OIT.

Apresentação Curiosa de um Tumor Cutâneo e o seu Sucesso Terapêutico

A doença de Bowen é um carcinoma espinocelular (CEC) in situ que pode evoluir para carcinoma espinocelular invasivo. Mulher, 61 anos, referia placa rosada, anular, bem delimitada, não descamativa, na região pré-auricular direita com 2 anos de evolução. Desde 2 meses antes da consulta, surgiu no seu centro um nódulo translúcido, com telangiectasias. A biopsia excisional do nódulo mostrou carcinoma espinocelular com doença de Bowen na periferia. A placa remanescente foi tratada com laser de CO2 seguida de terapêutica fotodinâmica com desaparecimento completo da lesão. Salienta-se este caso pela curiosidade da sua apresentação clínica e pelos bons resultados terapêutico e estético.

The Shading Sign: Is It Exclusive of Endometriomas?

OBJECTIVES: To investigate if the shading sign is an exclusive MRI feature of endometriomas or endometrioid tumors, and to analyze its different patterns. METHODS: Three hundred and fourty six women with adnexal masses who underwent 1.5/3-T MRI were included in this retrospective, board-approved study. The shading sign was found in 56 patients, but five cases were excluded due to lack of imaging follow-up or histological correlation. The final sample included 51 women. The type of tumor and the pattern of shading were recorded for each case. RESULTS: Thirty endometriomas and five endometrioid carcinomas were found. The remaining 16 cases corresponded to other benign and malignant tumors. The overall sensitivity, specificity, positive predictive value, and negative predictive value were 73%, 93%, 59%, and 96%, respectively. Restricting the analysis to cystic lesions without solid or fat component, sensitivity, specificity, positive predictive value, and negative predictive value were 73%, 96%, 94%, and 80%. Five shading patterns were identified: layering (15.7%), liquid-liquid level (11.8%), homogenous (45.1%), heterogeneous (11.8%), and focal/multifocal shading within a complex mass (19.6%). No significant correlation was found between these patterns and the type of tumor. CONCLUSIONS: The shading sign is not exclusive of endometriomas or endometrioid tumors. Homogenous shading was the most prevalent pattern in endometriomas and half of the cases with focal/multifocal shading within a complex mass were endometrioid carcinomas.

Cost-Effectiveness of Non-Vitamin K Antagonist Oral Anticoagulants for Atrial Fibrillation in Portugal

INTRODUCTION AND OBJECTIVES:Recently, three novel non-vitamin K antagonist oral anticoagulants received approval for reimbursement in Portugal for patients with non-valvular atrial fibrillation (AF). It is therefore important to evaluate the relative cost-effectiveness of these new oral anticoagulants in Portuguese AF patients. METHODS: A Markov model was used to analyze disease progression over a lifetime horizon. Relative efficacy data for stroke (ischemic and hemorrhagic), bleeding (intracranial, other major bleeding and clinically relevant non-major bleeding), myocardial infarction and treatment discontinuation were obtained by pairwise indirect comparisons between apixaban, dabigatran and rivaroxaban using warfarin as a common comparator. Data on resource use were obtained from the database of diagnosis-related groups and an expert panel. Model outputs included life years gained, quality-adjusted life years (QALYs), direct healthcare costs and incremental cost-effectiveness ratios (ICERs). RESULTS:Apixaban provided the most life years gained and QALYs. The ICERs of apixaban compared to warfarin and dabigatran were €5529/QALY and €9163/QALY, respectively. Apixaban was dominant over rivaroxaban (greater health gains and lower costs). The results were robust over a wide range of inputs in sensitivity analyses. Apixaban had a 70% probability of being cost-effective (at a threshold of €20 000/QALY) compared to all the other therapeutic options. CONCLUSIONS:Apixaban is a cost-effective alternative to warfarin and dabigatran and is dominant over rivaroxaban in AF patients from the perspective of the Portuguese national healthcare system. These conclusions are based on indirect comparisons, but despite this limitation, the information is useful for healthcare decision-makers.

Giant Angiolymphoid Hyperplasia with Eosinophilia on the Chest

Angiolymphoid hyperplasia with eosinophilia is a rare vascular proliferation characterized by single or multiple purplish, brownish papules and subcutaneous nodules, sometimes associated with pain or pruritus. This rare benign process occurs with a female predominance. Approximately 85% of the lesions occur in the skin of the head and neck; most of them are around the ear or on the forehead or scalp. Whether angiolymphoid hyperplasia with eosinophilia represents a benign neoplasm or an unusual reaction to varied stimuli, including trauma, the etiology remains unclear. Histopathologically, the lesions consist of a proliferation of blood vessels of variable size lined by large epithelioid endothelial cells and a variable inflammatory infiltrate of lymphocytes and eosinophils, sometimes with lymphoid follicle formation. The lesion is benign but may be persistent and is difficult to eradicate. We report on a case of a 58-year-old Caucasian man who presented a purplish pink dome-shaped tumor of size up to 8 cm in diameter located on the chest. We emphasize this case considering the unusual dimensions of the lesion (8 cm diameter) and the atypical location on the chest.

Identification of de Novo Germline Mutations in the HRPT2 Gene in Two Apparently Sporadic Cases with Challenging Parathyroid Tumor Diagnoses

The diagnosis of parathyroid carcinomas is often difficult. HRPT2 mutations have been identified in familial [hyperparathyroidism-jaw tumor (HPT-JT) syndrome] and sporadic parathyroid carcinomas, supporting that HRPT2 mutations may confer a malignant potential to parathyroid tumors. In this study, we report the clinical, histopathological, and genetic investigation of two unrelated cases, whom had apparently sporadic malignant parathyroid tumors, initially diagnosed as adenomas. In one case, the differential diagnosis was complicated by cervical seeding of parathyroid tumor cells. Genetic studies identified de novo HRPT2 germline mutations in cases 1 (c.518_521delTGTC [p.Ser174LysfsX27]) and 2 (c.226 C > T [p.Arg76X]), unveiling the hereditary HPT-JT syndrome in both patients. Furthermore, the identification of somatic mutations in the patients‟ parathyroid tumors provided evidence for complete inactivation of the HRPT2 gene, which was consistent with the tumor malignant features. The sensitivity of parafibromin immunostaining to detect HRPT2 mutations was limited. The present data suggests that patients with apparently sporadic parathyroid carcinomas, or parathyroid tumors with atypical histological features, should undergo molecular genetic testing, as it may detect germline HRPT2 mutations. Establishing the diagnosis of hereditary HPT-JT syndrome is relevant for clinical counseling and management of the carriers and their relatives.