Clinical and histopathology characteristics of Castleman disease: a multicenter study of 51 Brazilian patients.

Castleman's disease (CD) is a rare and heterogeneous lymphoproliferative disorder, with limited available clinical information in Brazil. A retrospective study was carried out through information contained in the medical records of 51 patients, between July 1999 and June 2020. Seven patients were excluded, and 44 were analyzed in total. The average age of unicentric CD (UCD) patients was 35 years old and of multicentric CD (MCD) patients was 49 years old (p = 0.013). Regarding gender, there was a predominance of females among patients with UCD (68.4%) and males in patients with MCD (57.9%) (p = 0.103). The most common site of involvement in UCD was the cervical region (36.8%). A total of 73.7% of patients with UCD and 68.4% of patients with MCD presented the histological form hialyne-vascular (HV) (p = 0.499). Most patients with laboratory abnormalities had MCD. A total of 78% of the patients were asymptomatic, with the majority of symptomatic patients with MCD (p = 0.042). Only two of the 27 patients evaluated for the presence of human immunodeficiency virus (HIV) had positive serology. HHV-8 was evaluated in 14 cases, being positive in two. Of the patients with UCD, 94.7% underwent excisional biopsy, against only 41.2% of patients with MCD (p = 0.01). The mean follow-up was 61 months. We observed similarities in the clinical profile between patients in our study and patients described in the literature, such as gender, mean age, B symptoms, visceromegaly, fluid accumulation, and treatment. Unlike the literature, the cervical region was the most affected site, besides the greater association of the HV histological subtype among patients with MCD.

Maxillary Sinus Natural Killer/T-Cell Extranodal Lymphoma Mimicking a Dentoalveolar Abscess.

Extranodal natural killer/T-cell lymphoma (ENKL) is an aggressive and infrequent malignant neoplasm. Early sinonasal ENKL clinical symptomatology is often not specific which can mimic several clinical odontogenic processes such as dentoalveolar abscesses. A 41-year-old female was referred to our institution due to facial pain with skin rash, fever, and intraoral swelling in the left side of the maxillary region. Computed tomography (CT) revealed a soft tissue hypodense area in the left side with bone discontinuity in anterior and lateral maxillary sinuses. Initial laboratorial tests showed no alterations on hemogram, coagulation profile, and immune phenotype (CD3+/CD4+). However, the lesion progressed fastly showing an evident growth, so it was decided that an intraoral biopsy should be performed. The diagnosis was sinonasal ENKLs. This case is an example that the rare T-cell lymphoma can mimic an odontogenic lesion, thus puzzling the clinician. Fortunately, the rapid growth of the lesion prompted the correct diagnosis and early treatment.

Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion.

Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Evolution is unpredictable and treatment depends on the extent and severity of the disease. It is described the case of a patient with various neurological symptoms, extensively investigated, who had its was diagnosed with histiocytosis from a single skin lesion. This report highlights the importance of Dermatology in assisting the investigation of difficult cases in medical practice.

Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion

Abstract: Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Evolution is unpredictable and treatment depends on the extent and severity of the disease. It is described the case of a patient with various neurological symptoms, extensively investigated, who had its was diagnosed with histiocytosis from a single skin lesion. This report highlights the importance of Dermatology in assisting the investigation of difficult cases in medical practice.

Small cell carcinoma of the bladder / Carcinoma de células pequenas de bexiga

Small cell carcinoma of the urinary bladder is an extremely aggressive and rare tumor. Even though small cell carcinoma most commonly arises from the lungs there are several reports of small cell carcinoma in extrapulmonary sites. Due to its low frequency there is no well-established management for this disease. We report the case of a 61 year-old man with small cell carcinoma of the bladder who underwent radical cystectomy following neoadjuvant chemotherapy. We also reviewed the literature for the optimal treatment strategy.

O carcinoma de células pequenas da bexiga urinária é um tumor extremamente agressivo e raro. Apesar desses tumores terem como sítio principal o pulmão, existem diversos relatos de carcinoma de pequenas células extrapulmonares. Pela baixa frequência, ainda não existe um tratamento bem estabelecido para essa neoplasia. Relatamos o caso de um homem de 61 anos de idade com carcinoma de células pequenas da bexiga urinária que foi submetido à quimioterapia neoadjuvante seguida de cistectomia radical. Fazemos ainda revisão na literatura em busca dos métodos de maior sucesso para o tratamento.

Small cell carcinoma of the bladder.

Small cell carcinoma of the urinary bladder is an extremely aggressive and rare tumor. Even though small cell carcinoma most commonly arises from the lungs there are several reports of small cell carcinoma in extrapulmonary sites. Due to its low frequency there is no well-established management for this disease. We report the case of a 61 year-old man with small cell carcinoma of the bladder who underwent radical cystectomy following neoadjuvant chemotherapy. We also reviewed the literature for the optimal treatment strategy.