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Pulmonary Clear Cell Tumor

Sugar Tumor


Presentation

  • Herein, we present a case of spindle cell subtype of pulmonary perivascular epithelioid cell tumor with prominent calcification and malignant potential in a 49-year-old woman.[ncbi.nlm.nih.gov]
  • Ultrastructurally, glycogen was present, mitochondria were abundant, and membrane-bound lamellated bodies consistent with premelanosomes were present in two cases, and equivocal in one.[nature.com]
  • Abundantly illustrated with over 1300 color images on 108 plates, the atlas presents not only the usual cytohistologic patterns of various disease entities, but also focuses on differential diagnostic problems and depicts the differentiating features.[books.google.com]
  • 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.[link.springer.com]
  • This malignancy remains clinically silent for most of its natural history, and patients therefore often present with advanced disease, with approximately 30% of patients presenting with metastases 5.[current-oncology.com]
Ascitic Fluid
  • False-positive immunostaining of normal epithelia and carcinomas with ascites fluid preparations of antimelanoma monoclonal antibody HMB-45. Am J Clin Pathol 1991; 95 : 454–459. 25 Tatum AH, Friedman HD. Letter to the editor.[nature.com]
Aspiration
  • The atlas includes techniques of bronchoscopy, brochoalveolar lavage, and fine needle aspiration biopsy, a detailed section on cytopreparatory techniques, liberal use of images on histomorphology to complement cytology, emphasis on diagnostic pitfalls[books.google.com]
  • Jpn J Surg, 1998, 28:985–987 CrossRef Google Scholar [17] Nguyen GK: Aspiration biopsy cytology of benign clear cell (“sugar”) tumor of the lung.[link.springer.com]
  • Bronchial biopsy and cytological aspiration were negative for malignancy. Subsequent fine-needle aspiration and biopsy of the left upper lobe gave a pathological diagnosis of lung metastasis of epitheloid clear cell sarcoma of the kidney.[archbronconeumol.org]
  • Metastasis to the thyroid diagnosed by fine-needle aspiration biopsy. Clin Endocrinol. 2005;62:236-241. Simoes MS, Asa SL, Kroll TG, et al. Tumours of the thyroid. In: DeLellis RA, Lloyd RV, Heitz PU, Eng C, eds.[path.upmc.edu]
  • ,Primary small cell anaplastic carcinoma of the breast diagnosed by fine needle aspiration cytology: a case report. Acta Cytol. 1998 Sep-Oct;42(5):1199-203.[breast-cancer.ca]
Persistent Cough
  • 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.[link.springer.com]
  • Symptoms of lung cancer are unspecific and dependent on anatomical site and include persistent coughing, hemoptysis, repeated pneumonia and signs of respiratory distress.[proteinatlas.org]
Rectal Bleeding
  • Case 4: A 40-year-old woman, who was otherwise healthy, presented with a recent history of profuse rectal bleeding.[nature.com]
Suggestibility
  • The size of the tumor and its Ki-67 index suggested malignant potential, and calcification was another rare characteristic. Immunostaining indicated that the tumor cells were positive not only for HMB-45 and Melan A, but also for CD34 and CD1a.[ncbi.nlm.nih.gov]
  • The occasional ultrastructural identification of melanosomes or premelanosomes ( 29, 31 ) suggests the reactivity is not spurious.[nature.com]
  • These findings suggest that patients with PEComas may require whole-body follow-up examinations because different subtypes of PEComas may occur multifocally.[keio.pure.elsevier.com]
  • Such data suggest that tumour behaviour may be, at least to some point, fixed very early in the disease process.[atlasgeneticsoncology.org]
  • Non-small cell carcinoma is suggested to originate from bronchogenic or alveolar cells and represents the most common form of primary lung cancer. Patient therapy is based on the tumour extent.[proteinatlas.org]
Confusion
  • One final neoplasm which could be confused with PEST’s is the recently described melanocytic clear cell neoplasm of the kidney ( 41 ).[nature.com]
  • PCCCL can be confused with metastatic hepatic lesions of renal, ovarian, or adrenal origin.[jtd.amegroups.com]
  • Often, symptoms are easily confused with common respiratory illnesses such as bronchitis or pneumonia, delaying an accurate diagnosis.[mdanderson.org]

Workup

  • Respiratory cytopathology is indispensable in the workup of patients suspected of having lung cancer requiring cytologic evaluation and is used increasingly in immunocompromised patients for the identification of infectious diseases.[books.google.com]
  • So, although all imaging modalities are helpful in the workup, biopsy is the only way to confirm the diagnosis The evolution of the case report we are presenting was rapidly lethal but some studies have reported the effectiveness of radiotherapy in the[ispub.com]
  • Staging workup begins with computed tomography of the lungs, as it is the most frequent site of metastasis.[dovepress.com]

Treatment

  • Generally asymptomatic or nonspecific pulmonary symptoms Diagnosis Generally incidental finding on imaging Requires tissue diagnosis with immunohistochemistry Radiology images Images hosted on other servers: CT of clear cell tumor of lung Prognosis and treatment[pathologyoutlines.com]
  • Small cell lung cancer is often staged in this way because it helps doctors decide if a patient might benefit from more aggressive treatments. Learn more about treatment options for small cell lung cancer.[cancer.net]
  • […] candidates for a second-line treatment approach.[clinicaltrials.gov]
  • We customize your treatment to deliver the most advanced, effective and least invasive treatments available for lung cancer.[mdanderson.org]
  • Renal cell carcinoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2010;21(suppl 5):v137–v139. 9. Pace K. Determining the best treatment for renal cell carcinoma in young patients.[dovepress.com]

Prognosis

  • Clinical features Generally asymptomatic or nonspecific pulmonary symptoms Diagnosis Generally incidental finding on imaging Requires tissue diagnosis with immunohistochemistry Radiology images Images hosted on other servers: CT of clear cell tumor of lung Prognosis[pathologyoutlines.com]
  • The prognosis of the condition is generally guarded, since the tumors are aggressive.[dovemed.com]
  • Prognosis The stage of small cell lung cancer and the patient’s overall health influence prognosis. Although small cell lung cancer is treatable at any stage, only some people with certain stages of small cell lung cancer can be cured.[cancer.net]
  • Prognosis Because most of the literature is retrospective and in the form of case reports, estimating mortality rates is difficult. In addition, not all reported cases are managed uniformly, thereby making it further difficult to estimate prognosis.[emedicine.medscape.com]
  • This new knowledge helps in the differential diagnostics of cancer, and in determining prognosis, but also provides an opportunity to better tailor existing therapies even to consider novel target agents.[ncbi.nlm.nih.gov]

Etiology

  • Generally arises in peripheral lung, rarely in trachea or bronchi Pathophysiology May rarely arise in patients with tuberous sclerosis along with lymphangioleiomyomatosis (LAM) and micronodular pneumocyte hyperplasia (MMPH) ( Am J Surg Pathol 1997;21:1242 ) Etiology[pathologyoutlines.com]
  • Modern Pathology - Primary Extrapulmonary Sugar Tumor ] Clear-cell tumor of the lung is a rare entity of unknown etiology and histogenesis. This neoplasm typically presents as an asymptomatic, peripheral, sharply rounded mass in the lung.[histopathology-india.net]
  • Smoking is accepted as the major risk factor, responsible for 70-90% of all cancer cases, although the etiology of lung cancer appears multifactorial with both environmental and genetic factors playing a role.[proteinatlas.org]
  • The etiology of EPSCC is unknown. While some authors have reported an association with tobacco smoking, others have not found a strong causative correlation with tobacco.[emedicine.medscape.com]
  • (Etiology) The exact cause of Large Cell Carcinoma of Lung is unknown.[dovemed.com]

Epidemiology

  • 1971;43:213 ) Considered a benign PEComa in the lung, as opposed to malignant PEComa ICD coding D14.31 Benign neoplasm of right bronchus and lung D14.32 Benign neoplasm of left bronchus and lung D14.30 Benign neoplasm of unspecified bronchus and lung Epidemiology[pathologyoutlines.com]
  • Renal Cell Carcinoma Epidemiology Renal Cell Carcinoma Symptoms Renal Cell Carcinoma Diagnosis Renal Cell Carcinoma Risk Factors[news-medical.net]
  • Epidemiology The age range is very wide, including children. Only very rarely is there an association with tuberous sclerosis Radiology Lesions are usually solitary and peripheral.[e-immunohistochemistry.info]
  • Epidemiology Approximately 1000 cases of extrapulmonary small cell carcinoma are reported annually in the United States, with an overall incidence of 0.1-0.4% of all cancers and 2.5–5% of all small cell carcinomas. [6] Global incidence of extrapulmonary[emedicine.medscape.com]
  • Pulmonary adenocarcinomas of the fetal lung type: a clinicopathologic study indicating differences in histology, epidemiology, and natural history of low-grade and high-grade forms. Am. J. Surg.[twmu.ac.jp]
Sex distribution
Age distribution

Pathophysiology

  • […] unspecified bronchus and lung Epidemiology Extremely rare, case reports and small case series Generally older adults 40 years old, may occur in children No clear gender predilection Sites Generally arises in peripheral lung, rarely in trachea or bronchi Pathophysiology[pathologyoutlines.com]
  • Pathophysiology Histologic criteria for diagnosis of extrapulmonary small cell carcinoma are same as those for pulmonary small cell carcinoma: the presence of uniform small cells with sparse cytoplasm, dense nuclei, and inconspicuous nucleoli. [2] Since[emedicine.medscape.com]

Prevention

  • How can Large Cell Carcinoma of Lung be Prevented? Currently, there is no known prevention method for Large Cell Carcinoma of Lung.[dovemed.com]
  • S, Tann A, Blanco AI, Paulino AC and Amato R: Spontaneous regression of thoracic metastases while progression of brain metastases after stereotactic radiosurgery and stereotactic body radiotherapy for metastatic renal cell carcinoma: Abscopal effect prevented[spandidos-publications.com]
  • It is anticipated that in the future, further detailed molecular investigations of gene expression in disease and normal tissues will lead to new prognostic, predictive, diagnostic, therapeutic and preventative tools.[atlasgeneticsoncology.org]

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