Mediastinal granular cell tumor arising from the left recurrent nerve; report of a case

Granular cell tumor is found in various organs but is rare in the mediastinum. We report a case of 36-year-old woman with a granular cell tumor in the left upper mediastinum. She was admitted to our hospital because of hoarseness. Laryngoscopic examination revealed left vocal cord paralysis. Chest computed tomography (CT) showed a 3.0 x 2.0 cm well circumscribed tumor at the left side of the trachea in the left upper mediastinum. As hoarseness was suspected to be attributable to the mediastinal tumor, tumor resection was performed. It was found that the tumor involved the left recurrent nerve. The tumor was completely excised with combined resection of the left recurrent nerve. Histopathologically, the tumor consisted of cells with eosinophilic granules and S-100 protein positive materials in the cytoplasm, and diagnosed a granular cell tumor.     

Retroperitoneal leiomyosarcoma found 5 cm in size: a case report

A 76-year-old woman presented with dull pain in left flank. Excretory urogram showed no function of left kidney. Retrograde pyelography, ultrasonography, computed tomography and magnetic resonance imaging demonstrated hydronephrosis of the left kidney and a mass lesion surrounding the ureter. The tumor was removed together with the upper region of the left ureter and kidney. The tumor was 5 x 3 x 2.5 cm in diameter and the pathological diagnosis was a leiomyosarcoma. Although recurrent tumor arose in the pelvis at 14 months after the operation, the patient has been in good general condition without showing any other evidence of recurrent lesion. This case may indicate the importance of early resection of retroperitoneal leiomyosarcoma.     

Surgically treated mucoepidermoid carcinoma in a 6-year-old boy; report of a case

A 6-year-old boy was admitted to our hospital with a history of recurrent obstructive pneumonia and hemoptysis. A chest computed tomography (CT) showed atelectasis in the left lower lobe. Angiograpy, which was performed for the suspicion of pulmonary sequestration, showed no feeding artery and revealed bleeding from the bronchial artery in the left lower lobe. As hemoptysis would not stop, an emergency left lower lobectomy was performed. Macroscopic examination of the resected specimen revealed a mass measuring 20 x 15 x 17 mm in the S8 proximal lung parenchyma, bronchiectasis, and an abscess in the distal lung parenchyma. Histopathologic examination determined the tumor was a mucoepidermoid carcinoma. Immunohistochemical staining revealed some tumor cells were positive for CA 19-9. The child has not had a recurrence 3 years postoperatively.     

Postoperative hypoglycemia after resection of pheochromocytoma: a case report

We report a case of severe hypoglycemia following resection of pheochromocytoma. A 39-year-old male was admitted to our hospital with a chief complaint of palpitation. Blood pressure and fasting blood glucose were within the normal range. Computed tomography and magnetic resonance imaging revealed a right adrenal tumor (7.5 x 5 x 7 cm) and 131I-MIBG scintigraphy showed marked tumor uptake of isotope. As plasma and urinary catecholamine levels were very high, a clinical diagnosis of pheochromocytoma was established. Doxazosin and propranolol were administered for 43 days prior to adrenalectomy. During intensive care monitoring 2 hours postoperatively, the patient became drowsy and began to sweat. Although blood pressure remained stable, severe hypoglycemia (38 mg/dl) and hyperinsulinism (63.67 microU/ml) were confirmed. Infusion of 50% glucose improved these symptoms and plasma glucose level and insulin secretion normalized within 15 days of surgery. We also reviewed 25 cases of hypoglycemia after resection of pheochromocytoma. We recommend close monitoring of blood glucose for at least 6 hours after adrenalectomy for pheochromocytoma.     

Thymic carcinoma (mixed small cell undifferentiated squamous cell carcinoma); report of a case

A 52-year-old man was admitted to our hospital on October, 1997 with complains of left anterior chest pain. A chest X-ray and computed tomography on admission showed evidence of a mass in the left anterior mediastinum. The patient was treated with combination chemotherapy [cisplatin (CDDP), etoposide (VP-16)] and radiation therapy (2 gray x 25 days), preoperatively. Median sternotomy revealed a tumor invading into the left anterior chest wall, the pericardium, and partial pleura. The tumor was extirpated with combined resection of invaded organs. Microscopically and immunohistochemically, the tumor was diagnosed mixed small cell and undifferentiated squamous cell carcinoma documented by Snover et al. The patient was discharge on March 1998, but 2 years later after operation, he was dead by recurrent. We reported a rare case of thymic carcinoma.     

Granulocyte colony stimulating factor-producing spindle cell renal cell carcinoma successfully treated by chemotherapy consisting of gemcitabine and doxorubicin

A 62-year-old female patient with a chief complaint of back pain and continuous fever was referred to our hospital. A computed tomography scan revealed a left renal tumor (76×54×67 mm) without a metastatic lesion. Laboratory examination showed an elevated white blood cell count of 23,200/μl. The patient underwent left radical nephrectomy and the histopathological diagnosis was spindle cell renal cell carcinoma (G3-4, pT4, pN2) with positive staining for granulocyte colony-stimulating factor. One month later, a computed tomography scan showed an enlarged locally recurrent tumor mass. Three cycles of combination chemotherapy consisting of gemcitabine (1,500 mg/m2, day 1) and doxorubicine (50 mg/m2, day 1) resulted in an 83% reduction of the tumor mass. A follow-up computed tomography scan after 2 weeks revealed rapid regrowth of the recurrent tumor. The patient died 199 days after the surgery. Combination chemotherapy consisting of gemcitabine and doxorubicin is a treatment option for patients with spindle cell renal cell carcinoma.     

Rare among the rarities--recurrent insulinoma

A patient with recurrent insulinoma without co-existing multiple endocrine neoplasia syndrome is described. In a 32-year-old man with recurrent episodes of symptomatic hypoglycemia, the supervised fast showed high insulin (24.5 IU/ml) and C-peptide level (3.06 ng/ml) with low blood sugar (27 mg/dl). A 1 x 1.5 cm nodule from the lower part of pancreatic body was removed on exploratory laparotomy. Histopathology confirmed the diagnosis of islet cell tumor. After 11 years, he started experiencing symptomatic hypoglycemic episodes with inappropriately elevated serum insulin and C-peptide levels (2.2 ng/ml). On pancreatic angiography, a 16 x 11 mm size tumor blush was noted. Due to fibrosis from previous surgery, distal pancreatectomy along with splenectomy was done. Histopathology confirmed the diagnosis of insulinoma. On both occasions, workup for multiple endocrine neoplasia turned out to be negative. He was given small amounts of intermediate acting insulin in early postoperative period, which was discontinued shortly thereafter.     

Mediastinal granular cell tumor: report of a case

Mediastinal granular cell tumor is rare. We report a case of 16-year-old woman with a granular cell tumor in the right upper-middle mediastinum. Chest computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 4.0 x 2.5 x 5.5 cm well circumscribed mass in the right upper-middle mediastinum. Tumor resection was performed. It was found that the tumor involved right vagus nerve. The tumor was completely excised with combined resection of the right vagus nerve peripheral to the right recurrent nerve. Histopathologically, the tumor consisted of round to polygonal cells with abundant eosinophilic granular cytoplasm, and diagnosed a granular cell tumor.     

Aneurysmal bone cyst arising in the rib; report of a case

We experienced with a relatively rare case of an aneurysmal bone cyst (ABC) arising in the left rib. A 34-year-old female, had experienced chest discomfort on the left anterior side and pain for 1 year. A chest X-ray suggested a left chest wall tumor involving the ribs. Computed tomography (CT), magnetic resonance imaging (MRI) and a bone scintigram revealed an expansive tumor of the anterior portion of the left 4th rib involving the 3rd and 5th rib with "blow out appearance" and "fluid-fluid level". Wide excision of the tumor and adjacent muscle tissue was performed with an antero-axillary incision. Chest wall reconstruction was performed with prolene mesh (140 x 90 mm). The resected specimen showed an encapsulated bony mass (75 x 60 x 35 mm) with multiple blood-filled spaces. Histopathological diagnosis was an ABC originating in the left 4th rib. She has been doing well with no evidence of recurrence 12 months postoperatively.     

Extraabdominal desmoid tumor of the chest wall

We report a rare case of desmoid tumor of the chest wall. A 75-year-old female visited our hospital due to a feeling of oppression in the left chest. A chest X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large mass in the left thorax. Surgical resection of the tumor was performed under the left standard thoracotomy. The tumor was 90 x 80 x 75 mm in size and diagnosed pathologically as desmoid tumor which showed proliferation of spindle shape cells and collagen fibers. Immunohistochemistry revealed that the tumor cells were positive for vimentin, alpha-smooth muscle actin and negative for CD34. The patient has been well without recurrence for 2 years and 2 months after surgery.     

Solitary fibrous tumor of the adrenal gland with renal cell carcinoma and angiomyolipoma at the same time; a case report

Solitary fibrous tumor (SFT) is a neoplasm of pleura and its occurrence in the retroperitoneal space is rare. We report a case of SFT of the adrenal gland associated with ipsilateral renal cell carcinoma (RCC) and angiomyolipoma (AML). A 48-year-old woman was referred to our hospital for a left renal AML. Computed tomography (CT) in our hospital showed a left adrenal mass (25 x 20 mm). Because the adrenal tumor was nonfunctioning, she was followed at outpatient clinic. Four years later, CT showed an increase in the left adrenal tumor size (42 x 30 mm) and a left RCC. Left adrenectomy and partial nephrectomy for RCC and AML were simultaneously performed. Histological examination revealed adrenal SFT and clear cell carcinoma and AML of the kidney. We present a brief review on histological characteristics of retroperitoneal SFT and its occurrence in the adrenal grand region.     

Metastatic adrenal carcinoma suspected as a primary tumor: a case report

A 62-year-old man was admitted with chief complaints of fever and general fatigue. Enhanced computed tomography revealed a tumor (5 cm in diameter) in the right suprarenal space, and the right renoportal lymph nodes were swollen. An abdominal TI-weighted MR image showed a low-intensity tumor measuring 4.5 x 5.5 x 6.0 cm. Chest computed tomography revealed two tumors. One was 1.5 cm, on the hilum of left lung, the other was 1 cm in the S6 lung field near the pleura. These findings strongly indicated primary adrenal carcinoma and lung metastasis. Right adrenalectomy was performed. Histological examination of this tumor revealed diffuse tumor cells with irregular nuclei, and it was diagnosed as poorly differentiated adenocarcinoma. There was no possibility of primary adrenal carcinoma. The primary site of the tumor was suspected to be the left lung.     

A case of paraureteral neurilemmoma causing ureteral obstruction: report of a case

Retroperitoneal neurilemmomas are rare tumors. They are usually asymptomatic, and often become quite large before they are discovered. A case is reported of a 75-year-old man with complaints of general malaise and left lumbar pain. Intravenous pyelography showed left renal nonfunctional and retrograde pyelography revealed the stenosis of the left ureter. The ultrasonogram demonstrated a cystic mass, and it was diagnosed as a retroperitoneal tumor with central necrosis by computerized tomography and magnetic resonance imaging. The tumor was removed with the left nonfunctioning kidney and ureter. The tumor was 3 x 3 x 4 cm, and the histological diagnosis was neurilemmoma (Antoni A + B type). There was a tight fibrous adhesion between the tumor and the left ureter, and the tumor was concluded to have originated from the adventitia of ureter. To date, 120 benign cases have been reported in Japan. They are reviewed and discussed.     

Giant cystic pheochromocytoma: a case report

The patient, a 59-year-old woman, was referred to our University Hospital for evaluation of a left mass. Ultrasonography revealed a left adrenal cystic mass. On excretory urograms, the left kidney was pressed downward by a suprarenal mass, and computerized tomography (CT) and magnetic resonance imaging (MRI) confirmed an adrenal cyst. 131I-meta-iodo-benzylguanidine (MIBG) scintigraphy showed prominent accumulation in the left adrenal mass and the capsule. Considering the elevation of catecholamines in both blood and urine samples, we performed a left adrenalectomy with a presumptive diagnosis of pheochromocytoma (tumor size: 11.6 x 7.5 x 6.5 cm, tumor weight 720 g). The subsequent pathological examination confirmed a left giant cystic pheochromocytoma. 131I-MIBG scintigraphy was the most useful tool in the diagnosis of the cystic pheochromocytoma.     

Schwannoma of the recurrent laryngeal nerve; report of a case

A 57-year-old woman was admitted to our hospital because of an abnormal shadow on her chest X-ray film and a palpable left neck mass. She had a mild cough. Computed tomography (CT) scans and magnetic resonance imaging (MRI) of the chest demonstrated a well-circumscribed, huge mass (approximately 14 cm in diameter) in the left anterior mediastinum. On June 12th, 2000, the mass was resected. During surgery, the tumor seemed to arise from the left recurrent laryngeal nerve. The pathological examination revealed the tumor as a schwannoma. Recurrent laryngeal nerve schwannoma is extremely rare.     

Castleman disease of the inter-lobar lymph node origin

We report a case of Castleman disease which originated from the inter-lobar lymph node, with a review of literatures. A 19-year-old woman complaining of cough was pointed out to have an abnormal shadow in the left lung field on chest X-ray. Chest computed tomography (CT) and magnetic resonance imaging (MRI) with enhancement revealed a homogeneous mass lesion at the left inter-lobar portion of the lung. Bronchoscopic findings demonstrated mucosal telangiectasis of the left lower bronchus. We performed the usual axillary thoracotomy and succeeded in extirpation of the tumor without large amount of bleeding. The tumor was elastic and hard, and 70 x 55 x 45 mm in size. Her postoperative course was uneventful and she was discharged on the 12th postoperative day.     

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST) complicated with von Recklinghausen's disease: a case report

A retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with von Recklinghausen's disease is reported. A 55-year-old woman was admitted with a left side abdominal mass. Physical examination showed numerous cafe-au-lait-spots, subcutaneous masses, scoliosis, and a baby's head-sized fixed mass in the left abdomen. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a 9 x 9 cm retroperitoneal mass. Two other tumors were also found. One on the left side of the T2-T3 thoracic spine, and the other posterior to the right hip joint. The retroperitoneal tumor was resected en bloc. The tumor was a solid yellow mass. Macroscopically it has a pseudocapsule of fibrous tissue, weighed 1,120 g and measured 9 x 9 x 15 cm. The histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST). Since the responsiveness of these tumors to chemotherapy and radiation therapy is poor, we did not administer adjuvant therapy. The patient is alive with no evidence of recurrence more than 6 months after surgey.     

A case of malignant fibrous histiocytoma arising from the renal capsule

A 62-year-old man was admitted with a chief complaint of general malaise. Computed tomography showed a large mass adjacent to the parenchyma of the left kidney. The mass was 17 x 13 x 12 cm in size. Preoperative diagnosis was left renal cell carcinoma and left radical nephrectomy was performed. Histopathologically, the tumor was diagnosed as malignant fibrous histiocytoma (MFH), and the tumor was considered to have arisen from the renal capsule. There has been no recurrence for 7 months postoperatively. We review 40 cases of MFH arising from the kidney or the renal capsule in the literature.     

Surgical removal of retroperitoneal liposarcoma after transarterial embolization: a case report]

A case of retroperitoneal liposarcoma that was removed after transarterial embolization is reported. A 62-year-old man was admitted with body weight loss and general fatigue. Computed tomography revealed an extrarenal tumor, 27 x 17 x 11 cm in size, in the left retroperitoneal space. Arteriography revealed that the hypervascular tumor was fed from the left renal artery, the left adrenal artery and the left lumber arteries (L1-L4). At first the patient underwent transarterial embolization of the left renal artery and the left lumbar arteries (L1, L3, L4). Twenty-two days later he underwent surgical excision of the tumor with combined resection of the left kidney and the descending colon. The resected tissue weighed 2,500 g. Histological examination revealed liposarcoma, pleomorphic type. His postoperative course was uneventful, and he has remained free of disease for 15 months.     

Huge retroperitoneal leiomyosarcoma: a case report

This is a case report of retroperitoneal leiomyosarcoma in a 61-year-old woman. She presented with a chief complaint of back pain. Computed tomography showed a left huge retroperitoneal tumor. The tumor was removed with left nephrectomy and left hemi-colectomy. Histological examination demonstrated leiomyosarcoma 26 x 20 x 16 cm in diameter and, 3.84 kg in weight. She died of local recurrence causing ileus 2 months after the surgery. Fifty-four cases of retroperitoneal leiomyosarcoma including the present case in the Japanese literature are reviewed.