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

Abstract

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.

Clear cell “sugar” tumor of the lung: Diagnostic characteristics of a rare pulmonary tumor: A case report and review of literature

“…Between the two genders, there is a slight female preponderance among the CCTL patients. [10,11] Although the age range of the patients have been reported from 8 to 63 years old with the median age of 57 years; the neoplasm seems to present with a higher incidence in the elderly. [12] Usually, the presentation is an asymptomatic patient with an isolated coin lesion, so the lesion often is discovered inadvertently by via radiologic.…”

“…Over a longterm observation and research, some unique presentations have been reported in literatures, such as the finding about concurrently with minute pulmonary meningothelial-like nodules in the patient with rectal adenocarcinoma, [13] or concurrently with thrombocytosis (platelet count > 1,000,000/mm³), which was solved after removing the CCTL. [11,14] Radiographically, CCTLs generally present as solitary, round, peripheral and parenchymal nodules with smooth boundary, but without evidence of cavitation or calcification. The location of the tumor has no lobar preference.…”

“…Over a long-term observation and research, some unique presentations have been reported in literatures, such as the finding about concurrently with minute pulmonary meningothelial-like nodules in the patient with rectal adenocarcinoma, [13] or concurrently with thrombocytosis (platelet count > 1,000,000/mm³), which was solved after removing the CCTL. [11,14]…”

Malignant lung PEComa (clear cell tumor): rare case report and literature review

“…CCTL has already been associated with other diseases, such as tuberous sclerosis complex ( 79 ), Birt-Hogg-Dube Syndrome, and essential thrombocythemia ( 26 , 27 ). However, in the malignant cases, it was not possible to determine the presence of risk factors strongly related to CCTL ( Table 2 ).…”