Localized amyloidosis is a rare condition characterized by the deposition of misfolding protein in a tissue, without other systemic manifestations. Only a small number of cases of localized amyloidosis of the tongue have been reported to date, in contrast to systemic amyloidosis, in which localization on the tongue is common. This study presents a rare case of localized amyloidosis of the tongue (amyloidoma) and provides a summary of the known literature of localized amyloidosis. This study describes the case of a 36-year-old female who presented with a swelling of the tongue base. The diagnosis of amyloidoma was made based on the findings of the physical examination, head and neck MRI findings and the histopathological examination with Congo red stain under polarized light. The histopathological diagnosis was as follows: Localized lambda light-chain amyloidosis. A thorough physical examination was performed by the ENT and Hematology/Oncology departments, without revealing signs of systemic disease. A series of hematological and imaging tests were also performed to verify that there was no sign of systemic involvement. The patient declined surgical excision and the 2-year follow-up did not reveal any changes in tumor dimension. Although the etiology of localized amyloidosis is yet not clear, the prolonged reaction of tissue plasma cells to environmental antigens may be a causative factor for the initiation of the neoplastic process.
RezumatLimfomul difuz cu celulă mare B mamar la bărbaţi reprezintă o entitate rară ce poate mima carcinomul mamar ca prezentare clinică, de aceea tratamentul iniţial pentru majoritatea pacienţilor rămâne cel chirurgical. Diagnosticul şi tratamentul precoce complementar, chimioterapia şi radioterapia, sunt foarte importante. Vom prezenta cazul unui bărbat de 56 ani diagnosticat cu limfom difuz cu celulă mare B, primitiv cu localizare mamară -formaţiune tumorală mamară fără limfadenopatie axilară. În urma biopsiei mamare, examenul histopatologic susţinut de testele imunohistochimice stabilesc diagnosticul de limfom difuz cu celula mare B mare primitiv cu pozitivitate pentru CD 20, CD 10, şi negativitate pentru BCL 2, myc şi BCL 6 cu index de proliferare foarte mare (Ki 67 90%). Pacientul a primit trei cure de chimioterapie R-CHOP (rituximab, ciclofosfamida, adriamicina, vincristine si prednisolon). Limfomul difuz cu celulă mare primitiv mamar la bărbat reprezintă o entitate foarte rară, fiind necesară o supiciune ridicată pentru diagnosticul şi tratamentul precoce.Cuvinte cheie: limfom difuz cu celule mare B, sân, bărbat, tratament
Molecular classification of endometrial carcinoma (EC) has represented a breakthrough ever since the TCGA (The Cancer Genome Atlas) project published it in 2013. Four distinct molecular subgroups were recognized based on somatic copy number alterations and tumor mutational burden: POLE (polymerase-epsilon) ultramutated, MSI (microsatellite instability) hypermutated (MSI-H), copy number low (CNL) and copy number high (CNH). However, implementing a standardized algorithm in current practice is far from being definitive. Our purpose in this study was to determine different molecular subgroups in a cohort of Romanian patients using cost effective and available immuno-histochemistry markers and outline statistical associations with different parameters. Tissue microarrays encompassing 50 cases with previously diagnosed ECs were tested for ER, PR, HER2, p53, MSH6 and PMS2 and results showed 68% MSI-H cases with statistical correlation with tumor size ] 2 cm (p=0.028) and no association with overall survival. CNH ECs were reported in 26% of cases and showed important statistical significance with TILs (p=0.041) and no correlations with overall survival. The CNL subgroup was reported in 74% of ECs cases and showed statistical significance with the histopathological subtype (p=0.006), pT (removal of primary tumor according to AJCC/UICC convention) (p=0.033), risk category according to ESMO (European Society of Medical Oncology) criteria (p=0.022) and ER expression (p=0.002). Five-year overall survival was 84%. Molecular classification is an important additional tool in current pathology guidelines for reporting ECs, but it is not currently standardized or available in all laboratories. Its importance in evolving treatment strategies for this disease is well documented so we strongly recommend routine testing for molecular prognostic factors.
L'impact de l'expression combinée de MYC, BCL2 et BCL6 sur la survie des patients atteints de lymphome diffus à grandes cellules B Introduction. C-MYC est l'un des facteurs de transcription essentiels qui jouent un rôle dans diverses fonctions cellulaires. Le réarrangement MYC est associé à une faible survie globale et une faible survie sans progression, un risque accru de rechute du système nerveux central chez les patients diagnostiqués avec un lymphome diffus à grandes cellules B (DLBCL) et traités par R-CHOP. En outre, l'amplification c-MYC est un facteur pronostique défavorable, amplifié par les réarrangements BCL2 et BCL6, respectivement, désignant les lymphomes de haut degré selon la révision de 2016 de l'OMS. Objectifs. Nous recherchons les corrélations de l'expression double ou triple des marqueurs c-MYC et
Retinal vasculitis is a complication associated with a local condition or it can be a retinal expression of a systemic inflammatory disorder, which initially may go unnoticed. Drug-associated vasculitis is frequently difficult to identify, because many patients follow treatments with more than one drug and the route of administration varies. A 35-year-old female patient presented with sudden hearing loss, headache and blurred vision that had started two weeks earlier and had become progressively worse. Ophthalmological examination revealed anterior uveitis, bilateral optic disc swelling and retinal vasculitis. The orbito-cerebral MRI, the CT scan and the serological tests were within normal limits. Unable to identify the cause of the retinal vasculitis, the patient's medical history was reviewed. The patient had recently had a gynecological procedure, where a 13.5 mg levonorgestrel intrauterine contraceptive device was implanted. After the device was removed and methylprednisolone treatment started, the patient presented a visible remission of the symptoms and signs. To the best of our knowledge, there is no case of retinal disease and optic disc edema associated with auditory problems caused by an intrauterine device. A proper examination, correlated with a very thorough medical history, could identify rare diseases and associations, in order to provide adequate medical care.
BACKGROUND. Primary sinonasal mucosal melanoma is a rare tumor with a poor survival rate. There is an inherent difficulty in diagnosing these lesions, especially because their complex anatomic locations and symptoms can be frequently confused with other benign or malignant processes. The purpose of our study was to report a difficult case and review the literature and recent research on therapeutic modalities. MATERIAL AND METHODS. We herein report a 61-year-old female patient, with a history of right eye enucleation and prosthesis, who presented with obstruction of the left nostril, anterior and posterior mucopurulent rhinorrhea, anosmia, left facial numbness, left exophthalmia accompanied by ipsilateral epiphora and decreased visual acuity. RESULTS. Clinical and imagistic testing revealed a large, grayish, fleshy tumor localized in the left maxillary sinus, with extension to the left orbit (producing osteolysis of the inferior and medial orbital walls), nasopharynx, ethmoidal cells and left frontal sinus. Pathological and immunohistochemical examination confirmed the diagnosis of mucosal melanoma. Other primary sites were excluded. The patient succumbed shortly after, following only palliative treatment. CONCLUSION. Early diagnosis of primary sinonasal mucosal melanoma is essential but very difficult to detect. Any symptoms such as unilateral epistaxis or nasal obstruction in a patient over the age of 60 should be rendered suspicious. Pathological and immunohistochemical examination for diagnosis and prognostic factors are important. Although surgery is the first option for treatment, one must consider, according to tumor staging, radiotherapy and chemotherapy with immunotherapy as a viable course of treatment for advanced cases.
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