The “sugar” clear cell tumor of the lung—clinical presentation and diagnostic difficulties of an unusual lung tumor in youth

Góra-Gębka, Magdalena; Liberek, Anna; Bako, Wanda; Szumera, Małgorzata; Korzon, Maria; Jaskiewicz, K

doi:10.1016/j.jpedsurg.2006.02.035

Cited by 25 publications

(15 citation statements)

References 4 publications

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“…Hemoptysis and high fever are rare presenting symptoms of CCTL. [7,8] Our patient CCTL radiographically presents as a round, peripheral parenchymal nodule with no evidence of cavitation or calcification. On CECT scans, they might show intense postcontrast enhancement, due to its rich vascular stroma.…”

Section: Discussionmentioning

confidence: 78%

PEComa of the lung

et al. 2010

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Perivascular epithelioid cell tumor (PEComa), also called clear cell ''sugar'' tumor of the lung, is a rare benign tumor arising from perivascular epithelioid cells (PECs). We report a case of a 15-year-old boy who presented with right lower lobe lesion which turned out to be a clear cell tumor of the lung. An [18F]-fluoro-2-deoxy-D-glucose (FDG) - positron emission tomography (PET) scan revealed mild FDG uptake in the lung lesion (SUV< 1) with no active uptake elsewhere in the body. We discuss the clinical, radiologic and immunohistochemical features of clear cell ''sugar'' tumor of lung and compare them with published literature.

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Section: Discussionmentioning

confidence: 78%

PEComa of the lung

et al. 2010

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“…Thrombocytosis may disappear with the treatment of the tumor 6. It has been reported in the “sugar” clear cell tumor of the lung an increased platelet count, but, unlike our case, the platelet counts became normal after the tumor treatment 7. Bone marrow biopsy was performed from our patient to examine bone, but JAK mutation assessment was not performed.…”

Section: Discussionmentioning

confidence: 58%

“…The diagnosis is usually more difficult with thin needle aspiration. Surgical excision is better for diagnosis and curative treatment 7-9. We preferred surgical removal for accurate diagnosis and curative treatment.…”

Section: Discussionmentioning

confidence: 99%

Lung Clear “Sugar” Cell Tumor and Jak V617f Positive Essential Thrombocythemia: A simple Coıncıdence?

Yazak

Sargın²,

Yavaşoğlu

et al. 2013

Mediterr J Hematol Infect Dis

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The primary clear cell tumor of the lung is an extremely rare benign tumor. This tumor is called “sugar tumor” since clear cell tumor of the lung contains abundant glycogen. We here present a case of lung clear cell tumor of the lung associated to essential thrombocythemia. To the best of our knowledge, there is no report about this association.A 44-Year-Old Woman admitted to our clinic with a 2-month history of fatigue. On physical examination, the spleen was 3 cm palpable below the left costal margin on the mid axillary line. The laboratory tests revealed an elevated platelet counts (1,014,000/mm3). A pulmonary nodule (3,5 cm) was detected in the upper right lobe on the chest X-ray. Then, thoracic computed tomography (CT) was planned. The nodule looked like benign pattern on CT scan and total excision was performed for curative and diagnostic treatment.Microscopically, the tumor was composed of nests of rounded or oval cells with distinct cell borders, optically clear cytoplasm and small nucleus. By immunohistochemistry, tumor cells were positive for HMB-45, NSE and focal S100 antigen. And, it was diagnosed as clear “sugar” cell tumor. After tumor excision the lasting thrombocytosis induced us to perform bone marrow biopsy and JAK2 mutation research.Diagnosis of Essential Thrombocythemia was made. In conclusion, it is important to make an evaluation for myeloproliferative diseases in clear “sugar” cell tumor in adults if thrombocytosis was lasting after treatment.

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“…According to World Health Organization guidelines, excision is the treatment of choice for CCTLs and no adjuvant therapy is recommended. CCTLs have previously been reported to behave in a benign fashion, although one study reported a case of fatal recurrence and metastasis . Tumors with characteristics including size >2.5 cm, necrosis, a mitotic index in 1/50 high‐power fields, marked pleomorphism, and nuclear atypia could be considered as having malignant potential .…”

Section: Discussionmentioning

confidence: 99%

“…CCTLs have previously been reported to behave in a benign fashion, although one study reported a case of fatal recurrence and metastasis. 9 Tumors with characteristics including size >2.5 cm, necrosis, a mitotic index in 1/50 high-power fields, marked pleomorphism, and nuclear atypia could be considered as having malignant potential. 10 In our case, necrosis and a mitotic index were not found, but the tumor was 40 mm in diameter and the Ki-67 labeling index was 3-5%, which suggested malignant potential.…”

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confidence: 99%

Spindle cell subtype of pulmonary clear cell tumor with prominent calcification and malignant potential

et al. 2017

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Perivascular epithelioid cell tumor of the lung, also known as clear cell “sugar” tumor, is a rare benign tumor arising from perivascular epithelioid cells. 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. Histologically, the striking feature of this lesion was attributed to the presence of spindle cells arranged in a diffuse pattern, which is a pitfall for diagnosis. However, some of the lesion contained polygonal tumor cells with clear abundant cytoplasm surrounded by thin‐walled vascular spaces. 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. This tumor should be distinguished from tumors with rich spindle cells such as sarcoma, clear cell carcinoma, or metastatic tumors. The patient in this case was alive with no tumor recurrence or metastasis six months after lobectomy.

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The “sugar” clear cell tumor of the lung—clinical presentation and diagnostic difficulties of an unusual lung tumor in youth

Cited by 25 publications

References 4 publications

PEComa of the lung

PEComa of the lung

Lung Clear “Sugar” Cell Tumor and Jak V617f Positive Essential Thrombocythemia: A simple Coıncıdence?

Spindle cell subtype of pulmonary clear cell tumor with prominent calcification and malignant potential

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