A 44-year-old woman was admitted to our clinic with a 2-month history of fatigue, cough, dyspnea and fever with hemoptysis. There was not familiarity for lung tumor or tuberculosis. On pathological examination, the spleen was 3 cm palpable below the left costal margin on the mid axillary line. The physical examination of other organs was normal. The results of laboratory tests showed hemoglobin level of 12, 2 g/dl, hematocrit 37,5%, white blood cell count 10,200/mm3, platelet count 1,014,000/mm3. The peripheral blood smear demonstrated normochromic normocytic red blood cells, an increased platelet number, a normal differential leucocyte count with 70% neutrophil, 2% eosinophil and 28% lymphocyte.
The laboratory parameters were as follows: Iron (Fe): 49 ug/dl, transferrin: 210 ug/dl, Fe saturation: 16%, UIBC: 252 ug/dl, TIBC: 301 ug/dl, VitB12: 228 pg/ml, and ferritin: 88 ng/ml. Urine analysis revealed normal findings. Sedimentation rate was 18 mm/h; C-reactive protein was 5 ng/ml.
A pulmonary nodule (3,5 cm) was detected in the upper right lobe on the chest X-ray. Then, thoracic CT was planned. Thoracic CT revealed a solid peripheral subpleural nodule in the right upper lung lobe apical segment with diameter of 37 mm. Nodule has homogeneous pattern showing umbilication in lobule contour-good bordered medial part (Figure 1).
Abdominal CT scan, performed to exclude metastatic renal cell carcinoma, showed only mild splenomegaly. 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 nuclei. By immunohistochemistry, tumor cells were positive for HMB-45, NSE and focal S100 antigen. The immunohistochemical staining for synaptophysin was negative. And, it was diagnosed as clear "sugar" cell tumor.
Malignant features of Clear "Sugar" Cell Tumor were excluded by imaging investigations before and after surgical tumor treatment.
However, considering that thrombocytosis continued after the surgical removal of the clear cell tumor in the third month in absence of infections, bone marrow biopsy was performed. The cellularity was 70% and the myeloid/erythroid series ratio was 4. Megakaryocytic series was hyperplasic. There were no atypical cells. Iron stores were normal. Normal karyotype was provided by GTL banding (G banding). JAK V617F heterozygote mutation was detected by polymerase chain reaction (PCR). Essential thrombocythemia was diagnosed according to the World Health Organization (WHO) criteria.
Being the platelet count over 1,000,000/mm3 in the 3rd month after the surgical intervention, the patient was started on therapy with oncocarbide. At present she is at 18th month of the follow-up in good condition with a normal abdominal and thoracic CT.
In CT parenchyma window in right lung upper lobe apical segment, a solid peripheral subpleural situated nodule which is 37 mm in diameter and has a homogeneous formation showing umbilication in lobule with a contour-good bordered medial part.