Ameloblastomas account for 9%-11% of all odontogenic tumors and usually arise from the mandible. Peripherally arising ameloblastomas are exceedingly rare, comprising 1%-2% of all ameloblastomas. We present a case of peripheral ameloblastoma with malignant differentiation in a 72-year-old male patient. The patient underwent a biopsy of the left palatoglossal fold, and histological examination of the specimen demonstrated a well differentiated squamous cell carcinoma in situ with at least superficially invasive growth and features suggestive of peripheral ameloblastic carcinoma. The patient chose radiation therapy as opposed to surgical excision.
Pleuroparenchymal fibro-elastosis (PPFE) is a rare idiopathic interstitial pneumonia. Secondary forms of PPFE may occur in patients following lung- or bone marrow transplantation. Here we report a middle-aged woman who presented with dyspnea and cough. She had been working as a cleaning woman in an agriculture company and logistic firm. Chest HRCT was highly suggestive for PPFE. Histopathological examination of a surgical biopsy showed a pattern of PPFE with airway-centered fibro-elastosis, constrictive bronchiolitis and birefringent particles in the pathological areas only. Using transmission electron microscopy with X-ray diffraction (TEM/EDX) these particles were identified as quartz and silicates, both components generally found in soil. The present case illustrates that secondary PPFE may result from agricultural dust exposure and stresses the need for a careful workup of the occupational history.
We reported a case of urethral adenocarcinoma in a 67-year-old woman who was admitted for difficulty voiding, irritative voiding symptoms, bloody urethral discharge and urinary incontinence. Physical examination was realised by gyneacologist and conclued to urological pathology. Urological examination was showed urethral ectropion which was bleeding. She had no fewer, no pelvic pain even less loss weight. Her general state was satisfactory. The lymph nodes of right side inguinal were felt. Computed imaging was showed involvement of right side bladder and bilateral iliac inguinal lymph nodes. Urethral ectropion biopsy anatomopathological screening was conclued to clear cell adenocarcinoma but CIS lesions were not found. The tumor staging was T3N2M0. A radiotherapy was perfomed. The patient was improvement of voiding symptoms and decrease lymph nodes size. Primary urethral carcinomas are rare pathology especially in the woman. Several histological types exist. However, chemotherapy and radiotherapy are very effective on very advanced stages. This cancer may be revealed by an urethral ectropion in a year-old woman.
The urachus is an embryological remnant that extends from the bladder to the umbilicus. Urachal neoplasms represent less than 1% of malignant bladder tumors. The new 2016 edition of disease classifications from the WHO has updated the classification of epithelial neoplasms of urachal origin, distinguishing between glandular and non-glandular neoplasms and between cystic and non-cystic adenocarcinomas. In this article, we present two cases with similar clinical presentations but differing histology. We address their morphological, immunohistochemical, and molecular characterization; review the classification and diagnostic criteria of glandular urachal neoplasms included in the most recent literature with the aim of clarifying their differential diagnosis as well as their prognostic implication; and, ultimately, better understand these infrequent biological entities.
Background: Acquired hemophagocytic lymphohistiocytosis (HLH) is an aggressive hyperinflammatory syndrome. Without prompt diagnosis and proper treatment, it can be life-threatening. HLH is commonly present in the setting of other autoimmune disorders, infection, organ transplantation, and malignancy. However, to our knowledge, HLH associated with monoclonal gammopathy of undetermined significance (MGUS) has not been reported.
Case presentation: A 67-year-old woman with an extensive history of MGUS and renal transplant presented with progressive fatigue, weight loss, intermittent fevers, splenomegaly, and pancytopenia. Serum protein electrophoresis with immunofixation identified a new monoclonal protein IgG lambda and a rapidly increasing pre-existing free kappa light chain. A bone marrow biopsy revealed focal clusters of kappa restricted plasma cells which comprised less than 10\% of marrow cellularity. Skeletal survey was negative for osteolytic lesions. She was also free of any end-organ damage. Histological examination showed a prominent increase in histiocytes and macrophages, many of which show erythrophagocytosis and lymphophagocytosis. Together with her clinical presentation along with a hyperferritinemia, a diagnosis of HLH was confirmed utilizing the criteria of the Histiocyte Society. The patient underwent a splenectomy. Prompt treatment with etoposide and high dose dexamethasone eventually stabilized the patient and resulted in a full recovery, which coincided with the disappearance of the serum monoclonal IgG lambda.
Conclusions: This report reveals a novel association of HLH with the progression of MGUS. Familiarity with this syndrome and its association with other conditions is indicated to ensure prompt diagnosis and appropriate patient treatment.
Hemosiderotic fibrohistiocytic lipomatous tumor (HFLT) is a rare and supposedly benign fibrolipomatous entity. Clinical and imaging features are not specific. So, histologic, immunohistochemical and molecular analyses are required for diagnosis. The clinic and pathologic aspects of this lesion are diversely reported. Moreover, its reactive or neoplastic origin is still under debate. This lesion should be treated by complete excision. It is mandatory to be aware of its tendency to harbor higher-grade lesions with aggressive outcome. We report a case of HFLT affecting the dorsum of the third right finger in a 52-year-old women, in order to describe histologic patterns of this tumor and to discuss the current literature regarding the etiopathogeny and the cytogenetic features of this rare entity.