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Primary endobronchial amyloidosis: A rare case of endobronchial tumor.

ABSTRACT: Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8-37 months. The average life expectancy for primary tracheobronchial amyloidosis is approximately 9 years, so the early diagnosis of this disease is very critical to improve the prognosis of patients. We are presenting a case of 65 year old male which was initially diagnosed and treated as asthma exacerbation with minimal improvement. Further workup was done with CT chest, bronchoscopy and biopsy because of persistent shortness of breath, which revealed primary localized solitary endobronchial amyloidosis.

SUBMITTER: Lal A

PROVIDER: S-EPMC5925958 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Primary endobronchial amyloidosis: A rare case of endobronchial tumor.

Lal Amos A Akhtar Jamal J Khan Mohammad Saud MS Chen Yayan Y Yaron Goldman

Respiratory medicine case reports 20180226

Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8-37 months. The averag ...[more]

PMID: 29719808

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Bronchial rupture following endobronchial blocker placement: a case report of a rare, unfortunate complication.

Project description:BackgroundLung separation may be achieved through the use of double lumen tubes or endobronchial blockers. The use of lung separation techniques carries the risk of airway injuries which range from minor complications like postoperative hoarseness and sore throat to rare and potentially devastating tracheobronchial mucosal injuries like bronchus perforation or rupture. With few case reports to date, bronchial rupture with the use of endobronchial blockers is indeed an overlooked complication.Case presentationA 78-year-old male patient with a left upper lobe lung adenocarcinoma underwent a left upper lobectomy with a Fuji Uniblocker® as the lung separation device. Despite an atraumatic insertion and endobronchial blocker balloon volume within manufacturer specifications, an intraoperative air leak developed, and the patient was found to have sustained a left mainstem bronchus rupture which was successfully repaired and the patient extubated uneventfully. Unfortunately, the patient passed on in-hospital from sepsis and other complications.ConclusionBronchial rupture is a serious complication of endobronchial blocker use that can carry significant morbidity, and due care should be exercised in its use and placement. Bronchoscopy should be used during insertion, and the volume and pressure of the balloon kept to the minimum required to prevent air leak. Bronchial injury should be considered as a differential in the presence of an unexplained air leak.

| S-EPMC8404020 | biostudies-literature

Calcifying nested stromal-epithelial tumor of the liver: Case report of a rare primary liver tumor.

Project description:Calcifying nested stromal epithelial tumor is a very rare primary liver tumor in children. To our knowledge, few cases have been reported in literature. We describe the imaging appearance and histopathologic features of this tumor incidentally detected in a 2-year-old girl. This tumor should be considered in the differential when a large heterogeneous liver tumor with central scar and coarse/chunky calcifications is identified at imaging in the absence of elevated alpha-fetoprotein in a child.

| S-EPMC8258647 | biostudies-literature

A rare case of endobronchial mucoepidermoid carcinoma of the lung presenting as non-resolving pneumonia.

Project description:BackgroundMucoepidermoid carcinoma (MEC) is the most common malignant salivary gland tumor, and MECs of the lung are rare, accounting for 0.1-0.2% of malignant lung tumors. Pulmonary MECs are commonly found in the segmental or lobar bronchi, rarely presenting as endobronchial lesions.Case presentationHere we describe the case of a 21-year-old female with no comorbid conditions who presented at the emergency room with a cough, yellow phlegm, pleuritic chest pain, and a subjective fever. These symptoms had been present for approximately one week prior to the patient's arrival at the hospital. A chest X-ray revealed right lower lobe alveolar infiltrate and computed tomography of the chest showed dense consolidation of the right lower lobe with ovoid intraluminal density in the right main stem bronchus. Upon fiber optic bronchoscopy, an endobronchial lesion was found in the right main stem sparing the right upper lobe uptake. Endobronchial biopsy results was consistent with MEC of the lung. The patient underwent a bilobectomy with complete resection of the tumor.ConclusionEndobronchial MEC is a rare type of salivary gland tumor. Patients with low-grade MECs have a good prognosis, whereas those with high-grade MECs, which are aggressive and associated with metastatic disease, have a poor prognosis. However, early identification and surgical resection can result in a good prognosis.

| S-EPMC6115606 | biostudies-literature

Bronchoscopic management of a primary endobronchial salivary epithelial-myoepithelial carcinoma: A case report.

Project description:Here, we discussed a 55 y/o African man who recently immigrated from Nigeria to the United States and who presented to Parkland Memorial Hospital with a productive, intermittent cough of one year duration. The cough was associated with shortness of breath and chest pain. Cough was not associated with voice hoarseness, hemoptysis, melanoptysis, and wheezing. He had a computed tomography (CT) scan of the chest that showed a 1.9 cm mass in the right main stem bronchus with ipsilateral right lower lobe consolidation and bronchiectasis. The patient was seen by pulmonology who recommended bronchoscopy for diagnosis and possible intervention. Bronchoscopy showed a 90% obstructing mass in the proximal right mainstem bronchus and bronchus intermedius. The mass was large and endobronchial, circumferential, exophytic, and polypoid. The decision was made to undergo bronchoscopic tumor ablation using electrocautery snare, argon plasma coagulation (APC), suction, and forceps. The tumor was successful ablated. Microscopic examination revealed eosinophilic ducts tightly coupled with a surrounding layer of clear cell myoepithelial cells and the diagnosis of epithelial-myoepithelial carcinoma (EMC) of the lung was made. The patient was discharged from the hospital with scheduled outpatient visits for monitoring of the carcinoma by pulmonology and thoracic surgery. Unfortunately, he was lost to follow up.

| S-EPMC7232110 | biostudies-literature

Primary acral amelanotic melanoma: A rare case report.

Project description:The aim of the present study was to present a rare case of primary acral amelanotic malignant melanoma (AMM). A 61-year-old man developed an aggressive tumor in the front part of the sole of his left foot, which continued to increase in size for >1 year. The biopsy results revealed epidermis loss, ulcer formation, and the presence of abundant allotropic tumor cells throughout the dermis, with deeply stained nuclei, light reddish cytoplasm and visible multinucleated giant cells with heterogeneous nuclear division. The tumor cells exhibited partial formation of nests and bundled distribution, and there were no observed pigment particles. The diagnosis was confirmed as AMM based on the findings of the histopathological examination and immunohistochemical staining for Ki67 (+++), Melan-A (+++), human melanoma black 45 (+), CD20 (-), cytokeratin (CK)7 (-) and CK5/6 (-).

| S-EPMC7484730 | biostudies-literature

Multiple chromoanasynthesis in a rare case of sporadic renal leiomyosarcoma: case report

Project description:Renal leiomyosarcomas (LMS) are extremely rare neoplasms with aggressive behaviour and poor survival prognosis. The most frequent somatic events in leiomyosarcomas are mutations in TP53, RB1, ATRX and PTEN genes, chromosomal instability and chromoanagenesis. By using chromosomal microarray analysis we identified monosomy of chromosomes 3 and 11, gain of Xp (ATRX) arm and three chromoanasynthesis regions (6q21-q27, 7p22.3-p12.1 and 12q13.11-q21.2), with MDM2 and CDK4 oncogenes copy number gains, whereas no CNVs or tumor specific SNVs in TP53, RB1 and PTEN genes were observed.

2020-06-04 | GSE151766 | GEO

A rare case of renal gastrointestinal stromal tumor.

Project description:Renal cell carcinoma (RCC) accounts for about 3% of adult cancers. RCC is the third most common cancer among the urogenital cancers, clear-cell RCC being the most common histological subtype. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract and are derived from interstitial Cajal cells (ICCs). The ICCs in the urinary system were first shown in 2003 in a study by Solari et al. However, there is no publication of a GIST of renal origin yet. Here we report a case of renal GIST that has not previously been published in the literature.

| S-EPMC6562833 | biostudies-literature

Intracranial Inflammatory Myofibroblastic Tumor: A Rare Case Report.

Project description:IntroductionInflammatory pseudotumor encompasses a broad range of non-neoplastic and neoplastic entities, including inflammatory myofibroblastic tumors (IMTs). Because it is a rare mesenchymal tumor of unknown etiology and pathogenesis, and its clinical symptoms and radiologic features are not distinctive, intracranial IMT could be misdiagnosed as other extra-axial tumors. Here, we present a case of intracranial IMT suspected to be a brain abscess.Case presentationIn this case, a 73-year-old woman presented headaches, nausea, and vertigo. Brain computed tomography (CT) and magnetic resonance imaging showed 4 × 3 cm sized oval rim-enhanced lesion on the left cerebellopontine angle. Considering the patient's history of otitis media and CT findings, we hypothesized that this lesion was a chronic brain abscess. The initial burr hole drain surgery was unsuccessful because there was no abscess, leading to a second radical excision surgery. Histopathological and immunohistochemical analyses eventually revealed a final diagnosis of intracranial IMT.ConclusionIntracranial IMT is a rare disease with unknown pathogenesis. Diagnosis primarily depends on histopathological and immunohistochemistry analyses. As observed in our case, this disease may be mistaken for meningiomas, solitary fibrous tumors, or chronic abscesses due to its rare occurrence.

| S-EPMC11250673 | biostudies-literature

A rare case of primary cardiac B cell lymphoma.

Project description:Primary cardiac lymphomas represent an extremely rare entity of extranodal lymphomas and should be distinguished from secondary cardiac involvement of disseminated lymphomas belonging to the non-Hodgkin's classification of blood cancers. Only 90 cases have been reported in literature. Presentation of cardiac lymphomas on imaging studies may not be unambiguous since they potentially mimic other cardiac neoplasms including myxomas, angiosarcoma or rhadomyomas and therefore require multimodality cardiac imaging, endomyocardial biopsy, excisional intraoperative biopsy and pericardial fluid cytological evaluation to establish final diagnosis.Herein we report the case of a 70 y/o immunocompetent Caucasian female with a rapidly progressing superior vena cava syndrome secondary to a large primary cardiac diffuse large B cell lymphoma (NHL lymphoma) almost completely obstructing the right atrium, right ventricle and affecting both mitral and tricuspid valve. The patient had no clinical evidence of disseminated disease and was successfully treated with extensive debulking during open-heart surgery on cardiopulmonary bypass and 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy (R-CHOP).

| S-EPMC4021862 | biostudies-other

A rare case of cardiac tumor in a child.

Project description:Pediatric cardiac tumors are rare and usually benign. An infectious etiology like tuberculosis invading myocardium and presenting as infiltrative mass is extremely rare. We present a case of a 15 month old girl with clinical feature of cardiac failure who had infiltrative multiple myocardial masses in echocardiogram. Advanced cardiac imaging by Cardiac Magnetic resonance imaging (MRI) helped in tissue delineation. Therapeutic trial of anti-tubercular drugs in view clinical suspicion of Tuberculosis resulted in complete remission of symptom and disappearance of the cardiac mass.

| S-EPMC5241855 | biostudies-other

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