PEComa of the lung (original) (raw)
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Clear cell “sugar tumor” of the lung: Diagnostic features of a rare pulmonary tumor
Respiratory Medicine Case Reports, 2018
Clear cell tumor of the lung (CCTL) is an extremely rare neoplasm with about 50 cases reported in the literature so far. CCTL belongs to a family arising from putative perivascular epithelioid cells and is otherwise named as "sugar tumor" due to its high cellular glycogen concentration. Due to its rarity, diagnostic features of this entity are not widely known and this usually leads to misdiagnosis. Herein, we report a case of benign CCTL, which was primarily evaluated intraoperatively by FNA cytology and then by a pathological examination of the resected tumor. The cytologic preparations were moderately cellular and showed multiple large, irregular, cohesive clusters of ovoid or spindle tumor cells. Cells had clear cytoplasm, showing positivity with the periodic acid-Schiff (PAS) staining method owing to the glycogen (sugar) content. The rapid cytologic report excluded the possibility of malignancy and a middle lobectomy along with an anterior upper segmentectomy was performed. Immunochemistry revealed a diffuse positivity for HMB45, MART-1, SMA and focally for desmin, while specimen was negative for pancytokeratin cocktail AE1/AE3, cytokeratin7, cytokeratin20 and EMA. These findings confirmed the diagnosis of a benign CCTL. Due to its rarity and similarity with other clear cell tumors of the lung, awareness of this entity, recognition of the cytomorphologic features and familiarity with the associated clinical features can help clinicians avoid certain pitfalls in the diagnostic process. Considering its benign course, unnecessary extensive lung resections may also be avoided thus permitting conservative management of these patients.
Clear cell “sugar“ tumor of the lung — case report
Central European Journal of Medicine, 2012
Background: The clear cell "sugar" tumor of the lung is an extremely rare benign mesenchymal tumor. Aim: To report a case of the sugar tumor and discuss diagnostic differentiation of the tumor. Case report: A 53-year female presented with persisting cough. A CT scan revealed a round, 10 mm nodule located within the right lower lobe. The nodule was easily removed during thoracotomy. On the gross examination, the tumor was well circumscribed, and had a homogenous grayish-white appearance on the cut surface. The tumor consisted of round and oval cells with abundant clear cytoplasm, showing PAS pozitive abundant glycogen granules, which were removed by diastase pre-treatment before further staining with PAS. Immunohistochemical studies revealed the tumor cells were positive for HMB-45, vimentin, S-100 protein and very few cells for CD-117. The tumor cells were negative for αSMA, CK-7, AE1/AE3, CD-10, chromogranin and TTF-1. Conclusion: Based on the clinical, pathohistological and immunohistochemical data, the diagnosis of the primary clear cell sugar tumor of the lung was established.
Annals of Diagnostic Pathology, 2007
Clear cell “sugar” tumor (CCST) of the lung is a very rare benign neoplasm believed to arise from epithelioid cells of perivascular differentiation. This tumor is typically diagnosed by thoracotomy. To our knowledge, only 2 case reports of CCST have been previously published: one case in which cytologic findings were subsequently confirmed histologically and ultrastructurally to be CCST, and one case of CCST diagnosed preoperatively by transbronchial core-needle biopsy. We here depict an additional case of CCST of the lung that was preoperatively diagnosed by computed tomography–guided transthoracic fine-needle aspiration biopsy and core-needle biopsy. We underscore the importance of cytologic and histologic correlation and the role of ancillary studies in differentiating this lesion from other primary or metastatic clear cell tumors of the lung.
PEComa (Clear Cell “Sugar” Tumor) of the Lung: A Benign Tumor That Presented With Trombocytosis
The Annals of Thoracic Surgery, 2009
the fifth and sixth decades. Most times the pulmonary AVF is single, abuts the pleura, and is found in the lower lungs. Classified as either simple or complex, up to 90% are the former, with all feeding arteries arising from a single subsegmental artery. Causes include hepatopulmonary syndrome, metastatic thyroid carcinoma, schistosomiasis, actinomycosis, necrotizing pneumonias, mitral stenosis, systemic amyloidosis, penetrating chest trauma, and surgery for lung resection or congenital cardiac conditions [5]. Pulmonary AVFs do not usually affect cardiac hemodynamics. The degree of right-to-left shunt and the potential for paradoxical embolism dictate the clinical manifestations [5]. Patients may present with an asymptomatic lung nodule on a chest roentgenogram or with one or more of a broad spectrum of symptoms, including epistaxis, dyspnea, and hemoptysis. Physical examination may reveal cyanosis, digital clubbing, or pulmonary vascular bruits. Complications are often neurologic, secondary to paradoxical emboli. These include transient ischemic attacks, strokes, brain abscesses, migraine headaches, and seizures. Other complications include hemothorax resulting from transpleural rupture, hemoptysis, polycythemia, anemia, and pulmonary hypertension. The diagnostic evaluation is centered on identifying its presence as well as determining treatment. Oxygen saturation studies nearly always demonstrate hypoxemia and an elevated shunt fraction (Ͼ5%) [5]. The chest roentgenogram is usually abnormal in symptomatic patients, and it may reveal single or multiple round or oval masses of uniform density, occasional lobulation, sharply defined borders, and tubular opacities representing feeding vessels [5]. Contrast-enhanced computed tomography is useful for diagnosis, and pulmonary angiography provides further anatomical detail for consideration of embolization. Contrast echocardiography, radionuclide perfusion lung scanning, and magnetic resonance imaging can also be helpful. Contrast echocardiography can detect a right-to-left shunt, but it does not provide anatomical detail. Perfusion lung scanning may reveal decreased pulmonary arterial perfusion of the involved lobe and unexpected radionuclide activity in the brain and kidneys. The pulmonary AVF can be seen on a magnetic resonance image, but computed tomographic angiography is preferred, due to its higher spatial resolution. Treatment of the pulmonary AVF encompasses either surgery or embolotherapy. An untreated pulmonary AVF may lead to considerable morbidity and mortality. Treatment is indicated when it is symptomatic, greater than 2 cm in diameter, or has feeding arteries at least 3 mm in diameter [5]. Surgical resection was the mainstay of treatment until the late 1970s. Percutaneous embolization using coils or balloons (when feasible) is now the treatment of choice, because it preserves lung parenchyma, is less invasive, and can be repeated if necessary [6]. A large or central AVF may not be suitable for embolization due to the high risk of distal embolization to the left-sided circulation. In our patient, we postulate that a fistula was created between the right interlobar pulmonary artery and an adjacent branch of the inferior pulmonary vein by the automated stapling device used during wedge resection of the coccidioma. This complication gave rise to exertional dyspnea, hypoxemia, and paradoxical embolism, and the fistula was identified on cardiac magnetic resonance imaging and computed tomographic angiography, and as it was too large to treat safely with embolization, so it was surgically resected. There are no other reports of an AVF after wedge resection of a lung lesion with a stapling device.
Clear cell "sugar" tumour of the lung: a case report
The Malaysian journal of pathology
We report a case of clear cell "sugar" tumour of the lung (CCTL) occurring in a 26-year-old lady. The patient was asymptomatic and the lesion was picked up in the course of a pre-employment medical examination. A well-defined 5 cm nodule in the right lower lobe was detected on routine chest X-Ray. Microscopical examination of the coin lesion showed clear cells containing abundant diastase-sensitive intracytoplasmic glycogen, as demohstrated with periodic acid-Schiff stains. Tumour immunoreactivity for HMB-45 and non-reactivity for cytokeratin support the histological diagnosis. To our knowledge, this is the first reported case of CCTL in Malaysia.
Fine needle aspiration cytology of clear cell sarcoma of the kidney with spindle cell pattern
Cytopathology, 2003
PEComa (clear cell ''sugar'' tumor) of the lung is a rare benign tumor of the lung probably arising from the perivascular epithelioid cells (PECs). We report a case of pulmonary PEComa arising from the periphery of the right lobe of a 64-year-old male. To our knowledge, this is the second case in the English literature diagnosed by fine needle aspiration biopsy. In this case report, the clinical, cytologic and immunohistochemical features clear cell ''sugar'' tumor of the lung are discussed and compared with the previously published literature. The differential diagnosis and methods for distinguishing the various clear cells lesions in the lung are discussed. Diagn. Cytopathol. 2008;36:89-93. '
Journal of Thoracic Oncology, 2007
Objective: Neuroendocrine (NE) lung tumors consist of typical carcinoid (TC), atypical carcinoid (AC), large cell neuroendocrine carcinoma (LCNEC) and small cell carcinoma (SCLC). The determinant of FDG uptake for NE lung tumors has not been well elucidated. The aim of the present study is to investigate the relationship of FDG uptake and glucose transporter type 1 (Glut-1) expression in NE lung tumors. Methods: NE lung tumor patients (n=32; age, mean ± s.d.=67.8±10y; male:female=28:4) who had underwent F-18 FDG-PET before treatment were enrolled. There were 1 TC, 3 AC, 5 LCNEC, and 23 SCLC. FDG uptake was represented by maximum standardized uptake value (maxSUV). The paraffin sections of the tumor tissues were immunostained for Glut-1 (Neomarker, 1:50). The relation of FDG uptake and Glut-1 expression was assessed by Pearson correlation analysis. Results: The maxSUVs of all NE lung tumors ranged from 0.6 to 29.5 (mean±s.d.=7.7±5.4), whereas percentage Glut-1 expression ranged from 0 to 100% (18±24%). MaxSUVs of all NE lung tumors were significantly correlated with percentage Glut-1 expression (r=0.6471, p=0.0001). In subgroup analyses, maxSUV was also significantly correlated with Glut-1 expression in SCLC (n=23, r=0.6189, p=0.0016) and in non-small cell NE lung tumors (n=9, r=0.7039, p=0.0343). The maxSUV and the percentage Glut-1 expression were 1.7±2.0 and 5.0±0.0%, 7.9±3.2 and 35.0±32.4%, and 7.6±3.7 and 12.6±14.1% for AC, LCNEC, and SCLC, respectively. MaxSUV of SCLC was significantly higher than that of AC (p=0.007), but percentage Glut-1 expression was not significantly different among AC, LCNEC, and SCLC (p>0.05). One TC case had a maxSUV of 29.5 and 100% Glut-1 expression. Conclusions: In NE lung tumors, the maxSUV on FDG-PET was highly correlated with Glut-1 immunostaining positivity. This result suggests that Glut-1 expression is one of the determining factors of FDG uptake in NE lung tumors.
Primary clear-cell carcinoma of the lung
Respiratory Medicine, 1993
IlltrOdUCtiOll Primary clear-cell carcinoma of the lung is defined as a malignant epithelial neoplasm composed on histopathological examination largely of optically clear non-mucin producing cells without evidence of squamous differentiation (1). It is classified by the World Health Organisation as a variant of large-cell carcinoma. The presence of a few clear cells is not sufficient to make the diagnosis as they are said to occur in up to 10% of large-cell carcinomas that elsewhere show evidence of squamous or glandular differentiation (2). There are few reports of clear-cell carcinoma in the English literature (24), and it is the purpose of this study to describe two further cases, and particularly to stress some of the practical diagnostic problems. Case Reports CASE 1