Clear Cell Tumor of the Lung. A Clinicopathologic, Immunohistochemical, and Ultrastructural Study of Eight Cases

“…Immunostaining involving vimentin, s-100, HMB-45, and CD56 has been reported as diagnostically important. 7,8 The prognosis for clear cell carcinoma of the lung is believed to resemble that of other non-small-cell carcinomas. Because this disease is rare and glycogen-rich cells exhibit various histological types, the prognosis may be more complicated.…”

Section: Casementioning

confidence: 99%

Clear cell carcinoma of the lung

Kitada

Ozawa

Sato

et al. 2010

Gen Thorac Cardiovasc Surg

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We present here in the case of a patient who underwent resection of clear cell carcinoma of the lung, a rare histological type. A screening test of the 71-year-old woman revealed a 2.0-cm lesion in S4 of the right lung with a diagnosis of bronchioloalveolar carcinoma before resection. Under the guidance of video-assisted thoracoscopy, right middle lobe resection and mediastinal lymph node dissection were performed. The histopathological examination showed clear, slightly acidophilic tumor cells rich in fine granular components proliferating in an alveolar fashion. Immunostaining was diagnostically useful, distinguishing clear cell carcinoma from lung metastasis of renal clear cell carcinoma or clear cell squamous cell carcinoma.

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“…PEComas include angiomyolipoma (AML) of the kidney 4,13 and liver, 23 clear cell ''sugar'' tumor of the lung, [8][9][10] lymphangiomyomatosis (LAM), and lymphangiomyoma, 3,14,20 and a group of uncommon tumors that arise in soft tissue, visceral organs, and skin. 6,7,11,12,15,22,24 The most common primary sites of PEComa are the mesentery, omentum, gastrointestinal tract, uterus, and retroperitoneum, 7,12 whereas somatic soft tissue and skin are less often involved.…”

mentioning

confidence: 99%

Sclerosing PEComa: Clinicopathologic Analysis of a Distinctive Variant With a Predilection for the Retroperitoneum

Hornick

Fletcher

2008

American Journal of Surgical Pathology

136

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PEComas (tumors showing perivascular epithelioid cell differentiation) are a family of mesenchymal neoplasms that include angiomyolipoma, clear cell "sugar" tumor of the lung, lymphangiomyomatosis, and a group of uncommon lesions that arise in soft tissue, visceral organs, and skin. We describe a distinctive variant of PEComa that shows extensive stromal hyalinization, a feature not previously described in these tumors. Thirteen PEComas with extensive stromal hyalinization were identified from a total of 70 cases of PEComa received between 1996 and 2006 (19%). All patients were women, with a mean age of 49 years (range, 34 to 73y). One patient had tuberous sclerosis. Ten tumors (77%) arose in the retroperitoneum (8 pararenal), and 1 each in the pelvis, uterus, and abdominal wall. Median tumor size was 9.5 cm (range, 4.5 to 28 cm). All except 2 were grossly well-circumscribed. The tumors were composed of cords and trabeculae of cytologically uniform bland epithelioid cells with palely eosinophilic, granular to clear cytoplasm and round nuclei with small nucleoli, embedded in abundant densely sclerotic stroma. Five tumors contained a spindle cell component, and 6 showed focally sheetlike areas. In all cases the tumor cells were focally arranged around blood vessels. All tumors lacked the delicate nesting vascular pattern typical of other PEComas. Mitoses ranged from 0 to 3/50 high-power field (mean 1) in all cases except 1. One tumor showed abrupt transition to areas with strikingly pleomorphic morphology, marked nuclear atypia, frequent mitoses (22/10 high-power field), and fascicular and nested architecture. This was the only case with necrosis. All tumors were immunopositive for desmin (usually diffusely) and HMB-45 (generally in scattered cells); 12/13 (92%) expressed smooth muscle actin, 11/12 (92%) caldesmon, 11/12 (92%) microphthalmia transcription factor (D5), and 3/13 (23%) melan-A. Only 1 (8%) was focally S-100 positive. All tumors were negative for epithelial membrane antigen, PAN-K, and KIT (CD117). Follow-up was available for 9 patients, ranging from 10 to 64 months (median, 33). One patient (whose tumor showed transition to high-grade malignant morphology) developed metastases to lung, liver, and abdominal wall. No other tumor has recurred or metastasized thus far. Sclerosing PEComa is a distinctive variant with a predilection for the pararenal retroperitoneum of middle-aged women. Sclerosing PEComas seem to pursue an indolent clinical course, unless associated with a frankly malignant component. Long-term follow-up will be required to confirm these findings.

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Clear Cell Tumor of the Lung. A Clinicopathologic, Immunohistochemical, and Ultrastructural Study of Eight Cases

Cited by 105 publications

References 0 publications

Perivascular epithelioid cell neoplasms: pathology and pathogenesis

Perivascular epithelioid cell neoplasms: pathology and pathogenesis

Clear cell carcinoma of the lung

Sclerosing PEComa: Clinicopathologic Analysis of a Distinctive Variant With a Predilection for the Retroperitoneum

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