A 43-year-old Malay man presented to our facility with a three-month history of fever, non-productive cough and weight loss. He was a chronic smoker and had no significant medical history. Results of a physical examination were unremarkable. A chest radiograph revealed a large right lower zone lung lesion, and a subsequent computed tomography (CT) scan of the thorax and abdomen revealed a large heterogeneously enhancing soft tissue mass in the right lower lobe of the lung with intra-cavitary extension into the left atrium via the right inferior pulmonary vein (Figure ). Transthoracic needle aspiration of this mass was suggestive of carcinoma. Surgery was performed for the resection of this mass; a right posterior lateral thoracotomy was performed, followed by a right lower lobectomy. The left atrium was opened at the inferior part of the superior pulmonary vein and the tumor resected with a small cuff of left atrium. The entire tumor and right lower lobe was delivered en bloc, and the left atrial defect subsequently patched. Histology demonstrated a high-grade clear cell sarcomatoid tumor, suggestive of metastatic clear cell renal cell carcinoma, a diagnosis specifically considered by the pathologist. On immunohistochemistry, the lesion was focally positive for epithelial membrane antigen (EMA), CD10 and vimentin, but negative for anticytokeratin CAM5.2, thyroid transcription factor-1 (TTF-1), smooth muscle actin (SMA), S100, HMB-45, Melan-A, Hepar and synaptophysin. However, as no renal lesion was evident on the CT scan (Figure ), a diagnosis of alveolar soft part sarcoma was considered. An additional extensive investigation did not reveal a primary lesion or any other metastatic lesions.\nThen, four months later, our patient developed a subcutaneous mass in his left loin. A CT scan of the abdomen confirmed a large 11 cm tumor occupying nearly the entire right kidney with involvement of the pelvicalyceal system and proximal ureter (Figure ). The tumor also extended into the right renal vein and the inferior vena cava, with a 2 cm soft tissue nodule was seen in the subcutaneous layer of the left flank. Further imaging of the thorax demonstrated multiple lung nodules, a large right pleural-based mass and an enlarged subcarinal lymph node. A bone scan was performed, and suggested involvement of the right humeral head and multiple thoracic vertebrae. Excision biopsy of the subcutaneous nodule was performed, and histology demonstrated a tumor morphologically similar to the initially resected lung lesion, suggestive of a high-grade clear cell renal cell carcinoma with sarcomatoid differentiation (Figure ). On immunohistochemistry, the tumor was strongly positive for vimentin, CD10, focally positive for epithelial membrane antigen, melan-A and negative for TTF-1, S100, inhibin and synaptophysin (Figure ) The positive vimentin and negative inhibin results weighed against the likelihood of an adrenocortical tumor.\nOur patient was given palliative first-line therapy of sunitinib, with initial best response of stable disease. After three cycles of sunitinib, the disease progressed; our patient declined any further therapy and he eventually died 13 months after his initial lung resection.
A 56-year-old female was admitted to our clinic with a complaint of multiple masses in her left neck region. The past medical history of the patient included a left nephrectomy for renal cell carcinoma (the pathological analysis revealed clear cell + chromophob cell carcinoma) in 2000, otherwise normal. A physical examination of the patient revealed multiple painless cervical masses at different lymphatic levels in the left cervical region [].\nLaboratory findings were as follows, hemoglobin:14 gr/dl, hematocrit: 41,1%, erythrocyte count: 5.180.000/ mm3, leucocyte count: 6.390 / mm3 (66.4% neutrophils, 23.9% lymphocytes, and 7.2% monocytes), and erythrocyte sedimentation rate 31 mm / 1 hour. Urine analysis was normal. The Brucella agglutination test, and the MONO, VDRL, ANA, ANTİ-DNA, ASMA, AMA, ASO, and CRP tests were normal. The thyroid function tests were normal. The scintigraphic bone scan imaging was negative for metastatic lesions. Thyroid ultrasonographic examination and high resolution Pulmonary imaging were also found to be negative for metastasis. An abdominal ultrasonograpic examination showed a nephrectomy scar on the left side, but was otherwise normal. Ultrasonographic examination of the neck detected five lymphadenopathies of different sizes with the greatest dimension of 3 × 3 cm. The magnetic resonance imaging (MRI) scan also confirmed the ultrasonographic findings, and post contrast scanning showed heterogen and noduler contrast. Pathological examination of the fine needle aspiration biopsy (FNAB) of the lymphadenopathy that was greatest in dimension showed renal cell carcinoma (RCC), clear cell type.\nIn December 2006, left functional neck dissection was performed. Totally 23 lymphadenopathies were counted in the surgical specimen. The greatest lymph-node metastasis appeared as a thick, irregularly walled cystic mass, with a minimally enhancing or dense surrounding reaction that effaced the adjacent tissue planes []. In the pathological examination of the dissected lymphadenopathies, it was found that a third of them in the jugulodigastric region were metastatic renal cell carcinoma []. Postoperative radiotherapy to the neck region was performed. In the second and fourth months after the second operation, she has no evidence of recurrence in the neck region.