Fibroma mole gigante vulvar: relato de caso / Giant soft vulvar fibroma: case report

Fibroma mole, ou pólipo fibroepitelial, é uma lesão de proporções geralmente reduzidas, de cor hiperpigmentada ou igual à da pele, localizando-se frequentemente na face, pescoço, tronco e regiões intertriginosas. É um tumor classificado como benigno e pode acometer tanto homens quanto mulheres em idade reprodutiva e depois da quarta década de vida. Ocorre principalmente em obesos, diabéticos e durante a gestação. Com menor frequência, podem alcançar dimensões que excedem 5 cm. Seu crescimento pode ser lento ou rápido e comumente são assintomáticos, mas podem promover sangramentos por conta de ulcerações decorrentes de traumas repetidos. Apresentamos neste relato um fibroma mole, gigante, de localização vulvar, com 11 cm de comprimento, 11 cm de largura e 5 cm de espessura, pesando 500 g.

Giant soft vulvar fibroma is a fibroepithelial polyp lesion with generally reduced proportions, with a hyperpigmented color or similar to that of the skin, frequently located on the face, neck, trunk and intertriginous regions. It is a tumor classified as benign, can affect both men and women, of reproductive age and after the fourth decade, mainly obese, diabetic and during pregnancy. However, less frequently, they can reach dimensions that exceed 5 cm, may have a slow or accelerated evolution. They are commonly asymptomatic, but bleeding may be present due to ulcerations resulting from repeated trauma. In the current study, we describe a giant soft fibroma with a vulvar location measuring 11 cm in length, 11 cm in width, 5 cm in thickness and weighing 500 grams.

Myxoid stroma is associated with postoperative relapse in patients with stage II colon cancer.

BACKGROUND: Fibrosis surrounding cancer cells has been shown to affect cancer cell metastatic behavior. The present study aimed to explore the utility of myxoid stroma as a predictive factor for postoperative relapse in patients with stage II colon cancer. METHODS: The present study retrospectively investigated 169 patients who underwent curative surgical resection of stage II colon cancer. The fibrotic stroma was classified according to Ueno's criteria, and the patients were divided into the myxoid (MY) group and the non-MY (NMY) group. We also recorded tumor budding (TB) and investigated the combination of MY and TB for postoperative relapse. Postoperative survival was also explored. RESULTS: Thirty-two (18.9%) patients had MY. MY was significantly associated with tumor budding (TB) and postoperative relapse (p <  0.001 and p <  0.001, respectively). The 5-year RFS rates in MY group and NMY group were 52.1 and 94.6% (p < 0.0001), and the 5-year OS rates in MY group and NMY group were 74.6 and 93.3% (p = 0.001). Multivariate analysis showed that both MY and TB were significant risk factors for postoperative relapse (p < 0.001 and p = 0.02, respectively), and that only TB was a significant risk factor for OS (p = 0.043). Furthermore, compared with patients with either one of MY or TB, patients with both MY and TB had postoperative relapse more frequently (11.4% vs. 53.8%). CONCLUSIONS: The present study suggests that MY is a predictive marker for postoperative relapse in patients with stage II colon cancer.

Uncommon complications of breast prostheses. / Complicaciones infrecuentes de las prótesis de mama.

Breast implants are associated with well-known common complications that have been widely studied, such as rupture and capsular contraction. However, the increasingly growing number of patients with breast implants has led to the increased likelihood of coming across less common complications; these include seromas or late infection; adenopathies in the internal mammary chain; granulomas in the capsule of the implant, which in some cases can extend beyond the fibrous capsule; desmoid tumors associated with the implants; and breast implant-associated large cell anaplastic lymphoma. This article aims to review the main uncommon complications associated with breast implants and to describe and illustrate their findings in different imaging techniques. Proper management of these complications is important; this is especially true of late seroma and the diagnosis of breast implant-associated large cell anaplastic lymphoma for their repercussions.

KRAS Mutations in Papillary Fibroelastomas: A Study of 50 Cases With Etiologic and Diagnostic Implications.

Papillary fibroelastoma (PFE) is an increasingly recognized cardiac tumor. Despite its prevalence, controversy exists as to whether it represents a reactive or neoplastic process due to histopathologic similarities with Lambl excrescences (LEs), an accepted reactive phenomenon. Recently, KRAS mutations were reported in a small collection of PFEs, but the incidence of mutations and conditions in which they arise in are unknown. Furthermore, the relationship between PFE and LE has yet to be investigated. Institutional archives were queried for cases of PFE (2001-2017). Paraffin-embedded tissue was microdissected for tumor isolation. Prospectively identified LEs (2018) were collected and wholly isolated. Extracted DNA underwent droplet digital polymerase chain reaction analysis of the most common KRAS mutations (codons 12/13 and 61). Relevant clinical information was abstracted from the medical record. Fifty-two PFEs were tested from 50 patients (32 women). The median patient age was 67 years. Seventeen (33%) PFEs harbored pathogenic variants in tested KRAS codons (12 in codons 12/13; 5 in codon 61). Mutations were mutually exclusive. No clinical or pathologic correlates differed significantly from cases without detectable pathogenic variants. No pathogenic mutation were detected in LEs (n=20; P=0.002). Herein, we report on the largest series of PFE tested for KRAS mutations and present the largest cohort of KRAS-mutant PFEs to date, providing evidence in support of the notion that at least a subset of PFEs represents neoplasia. Moreover, the lack of KRAS mutations in LEs provides evidence as to the separate etiology of this accepted reactive lesion.

Incidence of reactive hyperplastic lesions in the oral cavity: a 10 year retrospective study in Santa Catarina, Brazil / Incidência de lesões hiperplásicas reativas na cavidade bucal: estudo retrospectivo de 10 anos em Santa Catarina, Brasil

Abstract Introduction Reactive hyperplastic lesions develop in response to a chronic injury simulating an exuberant tissue repair response. They represent some of the most common oral lesions including inflammatory fibrous hyperplasia, oral pyogenic granuloma, giant cell fibroma, peripheral ossifying fibroma, and peripheral giant cell lesions. Objective The incidence of those lesions was investigated in an oral pathology service, and the clinical characteristics, associated etiological factors, concordance between the clinical and histopathological diagnostic was determined. Methods A total of 2400 patient records were screened from 2006 to 2016. Clinical features were recorded from biopsy reports and patients' files. Results A total of 534 cases of reactive hyperplastic lesions were retrieved and retrospectively studied, representing 22.25% of all diagnoses. The most frequent lesion was inflammatory fibrous hyperplasia (72.09%), followed by oral pyogenic granuloma (11.79%), giant cell fibroma (7.30%), peripheral ossifying fibroma (5.24%), and peripheral giant cell lesions (3.55%). Females were predominantly affected (74.19%), the gingiva and alveolar ridge were the predominant anatomical site (32.89%), and chronic traumatism was presented as the main etiological factor. The age widely ranges from the 1st decade of life to the 7th. Clinically, the reactive hyperplastic lesions consisted of small lesions (0.5-2 cm) and shared a strong likeness in color to the oral mucosa. The concordance between the clinical and histopathological diagnostic was high (82.5%). Conclusion Reactive hyperplastic lesions had a high incidence among oral pathologies. The understanding of their clinical features helps to achieve a clearer clinical and etiological diagnosis, and the knowledge of factors related to their development. This may contribute to adequate treatment and positive prognosis.

Resumo Introdução As lesões hiperplásicas reativas se desenvolvem em resposta a uma lesão crônica que estimula uma resposta acentuada de reparo tecidual. Elas representam uma das lesões orais mais comuns, inclusive hiperplasia fibrosa inflamatória, granuloma piogênico oral, fibroma de células gigantes, fibroma periférico ossificante e lesão periférica de células gigantes. Objetivo A incidência dessas lesões foi investigada em um serviço de patologia bucal e as características clínicas, os fatores etiológicos associados e a concordância entre os diagnósticos clínico e histopatológico foram determinados. Método Foram selecionados 2.400 registros de pacientes entre 2006 e 2016. As características clínicas foram registradas a partir de laudos de biópsia e dos prontuários dos pacientes. Resultados Um total de 534 casos de lesões hiperplásicas reativas foram recuperados e retrospectivamente estudados, representando 22,25% de todos os diagnósticos. A lesão mais frequente foi hiperplasia fibrosa inflamatória (72,09%), seguida por granuloma piogênico oral (11,79%), fibroma de células gigantes, (7,30%), fibroma periférico ossificante (5,24%) e lesão periférica de células gigantes (3,55%). O sexo feminino foi predominante (74,19%), a gengiva e a crista alveolar foram o local anatômico predominante (32,89%) e o traumatismo crônico foi demonstrado como o principal fator etiológico. A idade variou desde a 1ª década de vida até a 7ª. Clinicamente, as LHR consistiram em pequenas lesões (0,5 a 2 cm) que apresentaram uma forte semelhança de cor com a mucosa oral. A concordância entre o diagnóstico clínico e histopatológico foi alta (82,5%). Conclusão As lesões hiperplásicas reativas apresentaram alta incidência entre as patologias bucais. A compreensão das características clínicas ajuda na realização de um diagnóstico clínico e etiológico mais claro, bem como determinar os fatores relacionados ao seu desenvolvimento. Dessa forma contribui para um tratamento adequado e um prognóstico positivo.

Novel PTEN mutation in Cowden syndrome: case report with late diagnosis and non-malignant course.

Cowden syndrome (CS) is an infrequent genodermatosis caused by mutations in the phosphatase and tensin homolog (PTEN) gene in the majority of cases. As such, it belongs to the PTEN hamartoma tumor syndrome spectrum. This disease has a variable clinical expression characterized by the development of multiple hamartomatous tumors in different organs, usually during the second and third decades of life, and a high cumulative risk of several malignancies. We present a case of Cowden syndrome with late diagnosis presenting with a florid dermatological expression and multiple benign tumors, but no malignancies. A novel PTEN mutation was identified.

Desmoid-type fibromatosis arising in a bifid rib chest wall.

Desmoid-type fibromatosis is a rare soft tissue tumor and the chest wall is one of the common sites of its extra-abdominal occurrence. A bifid rib is one of the congenital rib abnormalities. We report a case of desmoid-type fibromatosis arising in a chest wall's bifid rib. A 42-year-old female complained of right chest pain without remarkable medical, traumatic, or familial history. Chest-computed tomography revealed a chest wall tumor located adjacent to a bifid costal cartilage of third rib. We performed chest wall resection of second and third ribs. Pathologically, the tumor was diagnosed a desmoid-type fibromatosis of the chest wall. We surmise mechanical stimulation due to the bifid rib may be related to the occurrence of the tumor. In case of desmoid-type fibromatosis without somatic gene mutation, traumatic history, wound, implants, or use of female hormonal agents, we should search also local congenital abnormality.

Incidence of reactive hyperplastic lesions in the oral cavity: a 10 year retrospective study in Santa Catarina, Brazil.

INTRODUCTION: Reactive hyperplastic lesions develop in response to a chronic injury simulating an exuberant tissue repair response. They represent some of the most common oral lesions including inflammatory fibrous hyperplasia, oral pyogenic granuloma, giant cell fibroma, peripheral ossifying fibroma, and peripheral giant cell lesions. OBJECTIVE: The incidence of those lesions was investigated in an oral pathology service, and the clinical characteristics, associated etiological factors, concordance between the clinical and histopathological diagnostic was determined. METHODS: A total of 2400 patient records were screened from 2006 to 2016. Clinical features were recorded from biopsy reports and patients' files. RESULTS: A total of 534 cases of reactive hyperplastic lesions were retrieved and retrospectively studied, representing 22.25% of all diagnoses. The most frequent lesion was inflammatory fibrous hyperplasia (72.09%), followed by oral pyogenic granuloma (11.79%), giant cell fibroma (7.30%), peripheral ossifying fibroma (5.24%), and peripheral giant cell lesions (3.55%). Females were predominantly affected (74.19%), the gingiva and alveolar ridge were the predominant anatomical site (32.89%), and chronic traumatism was presented as the main etiological factor. The age widely ranges from the 1st decade of life to the 7th. Clinically, the reactive hyperplastic lesions consisted of small lesions (0.5-2cm) and shared a strong likeness in color to the oral mucosa. The concordance between the clinical and histopathological diagnostic was high (82.5%). CONCLUSION: Reactive hyperplastic lesions had a high incidence among oral pathologies. The understanding of their clinical features helps to achieve a clearer clinical and etiological diagnosis, and the knowledge of factors related to their development. This may contribute to adequate treatment and positive prognosis.

Tumor Formation of Adult Stem Cell Transplants in Rodent Arthritic Joints.

PURPOSE: While imaging matrix-associated stem cell transplants aimed for cartilage repair in a rodent arthritis model, we noticed that some transplants formed locally destructive tumors. The purpose of this study was to determine the cause for this tumor formation in order to avoid this complication for future transplants. PROCEDURES: Adipose-derived stem cells (ADSC) isolated from subcutaneous adipose tissue were implanted into 24 osteochondral defects of the distal femur in ten athymic rats and two immunocompetent control rats. All transplants underwent serial magnetic resonance imaging (MRI) up to 6 weeks post-transplantation to monitor joint defect repair. Nine transplants showed an increasing size over time that caused local bone destruction (group 1), while 11 transplants in athymic rats (group 2) and 4 transplants in immunocompetent rats did not. We compared the ADSC implant size and growth rate on MR images, macroscopic features, histopathologic features, surface markers, and karyotypes of these presumed neoplastic transplants with non-neoplastic ADSC transplants. RESULTS: Implants in group 1 showed a significantly increased two-dimensional area at week 2 (p = 0.0092), 4 (p = 0.003), and 6 (p = 0.0205) compared to week 0, as determined by MRI. Histopathological correlations confirmed neoplastic features in group 1 with significantly increased size, cellularity, mitoses, and cytological atypia compared to group 2. Six transplants in group 1 were identified as malignant chondrosarcomas and three transplants as fibromyxoid sarcomas. Transplants in group 2 and immunocompetent controls exhibited normal cartilage features. Both groups showed a normal ADSC phenotype; however, neoplastic ADSC demonstrated a mixed population of diploid and tetraploid cells without genetic imbalance. CONCLUSIONS: ADSC transplants can form tumors in vivo. Preventive actions to avoid in vivo tumor formations may include karyotyping of culture-expanded ADSC before transplantation. In addition, serial imaging of ADSC transplants in vivo may enable early detection of abnormally proliferating cell transplants.

Gardener-associated fibroma: an unusual cause of upper airway obstruction.

We present the first case of upper airway obstruction secondary to a retropharyngeal Gardner-associated fibroma (GAF). A 16-month-old infant presented with a 3-month history of worsening dyspnoea and apnoeic episodes. Examination revealed stridor and left-sided retropharyngeal asymmetry. MRI demonstrated a mass in the retropharynx. Tracheostomy and pharyngeal biopsy under anaesthesia were performed, and histology confirmed a diagnosis of GAF. The mass was excised using a transcervical approach, and postoperative recovery was unremarkable. GAF is associated with Gardner's syndrome (GS) and familial adenomatous polyposis (FAP), both of which are associated with multiple colonic polyps and increased risk of colorectal malignancy. Subsequent testing for an APC mutation seen in GS and FAP was negative in our patient. The details of this unusual presentation of a rare disease are given in addition to a review of the literature.

Extraordinary Presentation of Elastofibroma Dorsi After a Thoracotomy Procedure.

Elastofibroma dorsi is a reactive pseudotumor of connective tissue, typically located in the infrascapular region. Awareness of this benign entity is crucial for radiologists, as well as clinicians, to avoid misdiagnosis and potential patient harm. In this report, we present clinical and imaging findings of an elastofibroma dorsi after a thoracotomy procedure.

Ex vivo resection and intestinal autotransplantation for a large mesenteric desmoid tumor secondary to familial adenomatous polyposis: A case report and literature review.

RATIONALE: The mesenteric desmoid tumor requires special attention and the most demanding treatment. PATIENT CONCERNS: Here we present a rare case of a large mesenteric desmoid tumor secondary to familial adenomatous polyposis (FAP) in a 34-year-old man accepted the ex vivo resection, and intestinal autotransplantation. DIAGNOSES: A 34-year-old man was referred to our department with a 6-year history of intermittent hematochezia without any other discomfort after undergoing partial colectomy in February 2013, and 5 endoscopic mucosal resections of colon polyps between May 2012 and July 2015 due to pathological diagnosis of FAP. A computed tomography scan showed a huge abdominal mass with indistinct boundary at the root of the mesentery. The adjacent organs were pushed and most of the superior mesenteric artery branches were infiltrated. INTERVENTIONS: An en bloc resection (R0 resection), and an ex vivo resection followed by intestinal autotransplantation was performed. OUTCOMES: The patient was discharged from the hospital on the 25th day after the operation, and was regularly followed up after surgery with abdominal ultrasonography and laboratory-biochemical tests every month, and serial CT scans every 3 months which showed no evidence of tumor recurrence, thrombus, intestinal obstruction or abdominal infection so far. LESSONS: An ex vivo resection and intestinal autotransplantation appear feasible for cases with pathological lesions involving the vessels at the root of mesentry, and represents an attractive alternative for the management of mesenteric desmoid tumors.

Experimental models of pancreatic cancer desmoplasia.

Desmoplasia is a fibro-inflammatory process and a well-established feature of pancreatic cancer. A key contributor to pancreatic cancer desmoplasia is the pancreatic stellate cell. Various in vitro and in vivo methods have emerged for the isolation, characterization, and use of pancreatic stellate cells in models of cancer-associated fibrosis. In addition to cell culture models, genetically engineered animal models have been established that spontaneously develop pancreatic cancer with desmoplasia. These animal models are currently being used for the study of pancreatic cancer pathogenesis and for evaluating therapeutics against pancreatic cancer. Here, we review various in vitro and in vivo models that are being used or have the potential to be used to study desmoplasia in pancreatic cancer.

Multiple sclerotic fibromas of the skin: an important clue for the diagnosis of Cowden syndrome.

Cowden syndrome is a rare autosomal dominant condition characterised by mucocutaneous hamartomas and, most importantly, predisposition to various extracutaneous benign and malignant tumours. This disorder is associated with a germline mutation in the phosphatase and tensin homologue gene, a tumour suppressor gene, located on 10q23 chromosome. The expressivity of this genodermatosis is highly variable, therefore many of the cases remain undiagnosed. Skin and mucous findings are very common in Cowden syndrome and may represent the initial clinical manifestation leading to the diagnosis. The authors describe a case of a 58-year-old man with multiple cutaneous sclerotic fibromas associated with a previously unrecognised Cowden syndrome.