Familial atrial myxoma in three generations: case report

Familial myxomas constitute 7 % of the total myxoma population exhibiting Mendelian autosomal dominant inheritance. We present a rare case of autosomal dominant benign familial myxoma occurring in three generations of the family operated successfully at our institute. A 40-year-old female presented with chest pain and palpitation, was diagnosed with left atrial myxoma, and was operated in 1993. After 2 years of surgery, the patient developed recurrent myxoma, and excision was done in 1995. A 22-year-old son complained of dyspnoea and chest pain. On evaluation, 5.6?×?2.6 cm right atrial myxoma attached to the roof of the right atrium was found, and the patient underwent myxoma excision in 2007. A 3-year-old granddaughter suffered from right hemiplegia. Echocardiography revealed left atrial myxoma 3 cm?×?2 cm pedunculated lesion causing diastolic obstruction of mitral inflow. Operated patients in all three generations and other siblings have been genetically screened. On genetic evaluation, all diseased had autosomal dominant single-gene defect or microdeletion. Atrial myxomas presented at earlier age successively and in more aggressive form in these three generations.     

Neurofibroma originated from the intrathoracic vagal nerve in a patient with von Recklinghausen's disease--a case report

A neurofibroma originated from the intrathoracic vagal nerve was presented. A 26-year-old male was found to have an abnormal shadow in the right chest filed on a routine examination. He was complicated with Von Recklinghausen's disease and showed multiple "cafe au lait" spots over his extremities and trunk but no subcutaneous mass. A block resection of the mass was carried for the mediastinal tumor. The tumor was arising from the right intrathoracic vagal nerve which located just below the origin of recurrent nerve and was measured 3 X 3 X 6 cm. Histological examination of the specimen revealed neurofibroma. His postoperative course was uneventful. A brief review in Japan was made on 22 cases collected from the literature.     

Chest wall metastasis of endometrial cancer: case report and review of the literature

Introduction: Endometrial cancer is the fourth most common female cancer and also distant metastases to the chest wall associated with these tumors are seen less common. Surgical treatment options for metastases of endometrial cancer are limited.

Case: A 57-year-old patient who underwent total abdominal hysterectomy?+?bilateral salpingo oophorectomy and received adjuvant chemotherapy for endometrioid-type adenocarcinoma of the endometrium and tuba is presented. The patient initially presented with pleural effusion 8 years ago and the tumor was detected while during etiologic screening. The patient had no problems after adjuvant chemotherapy and had been living in another city when she presented with a mass on the chest wall measuring 20?×?15?×?12?cm 8 years after the initial diagnosis and distant organ metastasis due to the first surgery was detected. The mass was first thought to be a sarcoma and the tru-cut biopsy revealed a metastatic lesion that was immunohistochemically similar to the first surgical specimen. The mass was resected with a wide radical excision and ‘no touch’ technique. The patient was then discharged with no postoperative complications.

Conclusion: Here in, a case with a distant organ metastasis due to an endometrioid-type adenocarcinoma is presented accompanied by literature data.     

A rare case of eccrine spiradenoma—treatment and management

A young male patient presented with multiple swellings on his chest and the nape of his neck. Physical examination revealed multiple small papulonodular swellings measuring 0.5?×?0.5 cm to 2?×?2 cm, that were soft without discharge with no surrounding skin changes or induration. Skin biopsy samples were diagnosed as benign adnexal neoplasm consistent with eccrine spiradenoma, trichoepithelioma, and cylindroma, i.e., Brooke–Spiegler syndrome. Having confirmed this to be a case of eccrine spiradenoma, surgical excision was performed and the raw area was covered with a split thickness skin graft taken from the right thigh and sutured over the raw area. The sternal lesion was circumferentially excised and the wound was primarily closed by Z-plasty. Surgical excision is considered the gold standard for the treatment of these cases, with low rates of recurrence. Around 50 such cases have been reported in the literature to date. Although eccrine spiradenomas are usually solitary and small, the findings in our case underscore the fact that a variety of presentations are possible. With strict clinical suspicion and histological criteria, the correct diagnosis can be achieved, especially when combined with pertinent clinical information and laboratory studies.Level of Evidence: Level V, therapeutic study.     

Castleman's disease arising from the chest wall

An 18-year-old man was referred to our hospital because of an abnormality on chest radiography. Computed tomography showed a sharply defined, 5 × 2 × 3?cm, densely enhanced mass in the left side of the chest wall. We performed tumor resection under video-assisted thoracoscopy, with minimal bleeding. Histologically, the tumor was shown to be Castleman's disease. This is the eleventh case of Castleman's disease arising from the chest wall reported over the last 20 years.     

MRI findings of lumbosacral metastasis from occult follicular thyroid cancer: report of a case

A 63-year-old female was admitted to our hospital with bowel and bladder incontinence. Magnetic resonance imaging (MRI) showed a 13 × 12 × 12 cm mass invading the posterior regions of the L4, L5, S1 and S2 vertebrae with broad paravertebral soft tissue invasion. A Tru-cut biopsy of the mass was performed. The histopathological examination revealed metastatic follicular carcinoma of the thyroid. Thyroid functional tests were within the normal limits. Thyroid sonography revealed a heterogeneous, ill-defined, 14 × 9 mm hypoechoic solid nodule in the right lobe of the thyroid gland. On thyroid scintigraphy, an area of focal hyperactivity was detected in the right lobe at the nodule localization. Total thyroidectomy was performed, and the primary tumor pathology was determined to be follicular thyroid cancer. To our knowledge, only a few cases of lumbosacral cord compression as the initial manifestation of follicular thyroid carcinoma have been reported in the literature. We aimed to discuss the MRI findings of tumors in this age group with lumbosacral localization.     

R0 resection and reconstruction for a large,rapidly progressive chest wall sarcoma

Background

Chest wall sarcomas are a rare group of soft tissue malignancies with variable presentations. Here we describe the definitive management of a large, rapidly progressing chest wall sarcoma arising from the pectoralis major muscle.

Case report

An obese 42-year-old African American male with multiple medical comorbidities presented with new onset right-sided chest pain and a palpable right chest mass. Initial CT chest demonstrated a 9x9x9cm necrotic mass arising from the pectoralis major. CT-guided core biopsy was positive for high-grade spindle cell neoplasm (positive for smooth muscle actin, desmin, S100, and CD31; negative for CD34, PAX8, and beta-catenin). Staging imaging 2?months later demonstrated growth of the mass to 21.4?×?17.8?×?13.7?cm. The patient underwent neoadjuvant chemoradiation with surveillance CT imaging demonstrating a stable tumor. Then he underwent wide local excision of the mass followed by delayed local myocutaneous flap reconstruction and skin grafting. Final pathology was R0 resection, 38x20x18 cm tumor with 70% gross necrosis. Microscopic examination confirmed high-grade sarcoma with smooth muscle differentiation. Final pathologic staging was Stage III G3 pT2bNxMx.

Conclusions

This patient presented with a rare, rapidly enlarging high-grade leiomyosarcoma of the chest wall without metastases or violation of the thorax. We describe the definitive management including a multidisciplinary team to manage a complex and rapidly progressive sarcoma of the chest wall.

     

Case Report on Giant Parathyroid Adenoma with Review of Literature

Symptomatic primary hyperparathyroidism is still predominant form in developing countries. Sumit Shukla et al. (2008) Most of these lesions are usually less than 2 cm in diameter with serum calcium levels between 11 to 13 mg/dl. Our case had 3.9?×?2?×?1.7 cm non palpable right inferior parathyroid adenoma extending into superior mediastinum, serum calcium 17 mg/dl and vomiting as presenting symptom a case report.     

Enterovesical fistula caused by non-Hodgkin’s lymphoma of the ileum: report of a case

We herein present a rare case of enterovesical fistula caused by ileal non-Hodgkin’s lymphoma. A 75-year-old Japanese male presented with macrohematuria at Kosei General Hospital in December 2010. An egg-sized mass was palpable in his right lower abdominal region, and computed tomography (CT) revealed that the ileal tumor had invaded the right posterior wall of the urinary bladder (UB). A histopathological examination of a CT-guided needle biopsy specimen revealed diffuse large B-cell lymphoma involving the ileum and the UB. Thereafter, fecaluria appeared. A transurethral catheter was put in place, and there were no symptoms of cystitis. The patient received chemotherapy for the lymphoma, which produced a partial response. However, the fecaluria continued, and an examination of the small intestine with contrast revealed a thick and irregular wall of the ileum and a fistula between the ileum and UB. A partial resection of the ileum and a partial cystectomy were carried out in April 2011. The surgical specimen demonstrated two tumors 5 cm apart in the ileum, measuring 4.5 × 7 and 4 × 3 cm in size. The proximal tumor had directly invaded the UB and formed an ileovesical fistula. The patient made a good recovery and was doing well 5 months after the surgery without any evidence of recurrence.     

“A 40-year-old female with painless,slow growing prepatellar mass”

A 40-year-old woman from India presented with a mass in the front of her left knee which had been present for 8 months. Local examination revealed a globular mass of approximate size 5 cm × 4 cm × 4 cm in front of the lower pole of left patella. The patient was investigated with imaging studies and laboratory tests. Plain radiograph of the chest was normal. In addition, contrast enhanced Magnetic Resonance Imaging (MRI) of the left knee was performed. Based on the history, physical examination, laboratory and imaging studies, what is the differential diagnosis?     

Giant cell tumor of the sternum

A primary giant cell tumor (GCT) originating from the sternum is extremely rare. We report a case of a GCT originating from the sternum in a 45-year-old man who was referred to us for a mass in the anterior chest wall that had been growing slowly. Computed tomography revealed a soft tissue mass involving a large osteolytic and destructive lesion of the sternum body. Subtotal sternectomy and reconstruction with methylmethacrylate were performed. The tumor was 8.5 × 4.5 × 2.5?cm, and the histopathological examination confirmed GCT. Radical wide resection of primary sternum tumors and reconstruction with an appropriately rigid prosthetic material are necessary to minimize local recurrence.     

Use of contrast-enhanced computed tomography for management of a late-onset spontaneous massive chest wall hematoma

This is a rare case of a 68-year-old woman who was rehospitalized after uneventful redo double-valve surgery. An 8.3 × 12.9 × 16.4 cm tense right chest wall hematoma was diagnosed. This was precipitated by a single cough. Contrast-enhanced computed tomography revealed a bleeding source. Hematoma evacuation and hemostasis following emergency warfarin reversal produced an excellent outcome.     

Castleman''s disease arising from the chest wall

An 18-year-old man was referred to our hospital because of an abnormality on chest radiography. Computed tomography showed a sharply defined, 5 × 2 × 3 cm, densely enhanced mass in the left side of the chest wall. We performed tumor resection under video-assisted thoracoscopy, with minimal bleeding. Histologically, the tumor was shown to be Castleman's disease. This is the eleventh case of Castleman's disease arising from the chest wall reported over the last 20 years.     

A rare case of synchronous association of chromophobe renal cell carcinoma with urothelial carcinoma of urinary bladder

Introduction

Chromophobe renal cell carcinoma accounts for 3–5% of all RCCs. However, its association with urothelial carcinoma of urinary bladder has never been reported. We report a case of synchronous association of chromophobe RCC with low grade urothelial carcinoma of urinary bladder.

Ileocolic Intussusception Caused by a Giant Ulcerating Lipoma of Bauhin’s Valve: an Unusual Cause of Intestinal Obstruction in the Adult

We report a case of intestinal obstruction in a 73-year-old woman caused by ileocolic intussusception. The underlying cause was a giant submucosal ulcerating lipoma (6?×?3.3?×?3.8 cm) extending from the superior mucosal lip of Bauhin’s valve. Abdominal ultrasonography showed a pathognomonic target-like mass appearing as multiple concentric rings. CT scan confirmed the diagnosis. Because of apparent obstruction, a laparotomy with right hemicolectomy and ileocolic anastomosis was performed. Lipomas most frequently occur in the cecum and ascending colon where they represent the most common submucosal mesenchymal tumor. They typically occur in elderly women with an incidence that varies from 0.15 to 0.56 %. Pain, rectal bleeding, and obstruction are typical symptoms. When faced with an intussusception in children, reduction with air per rectum can be performed. In adults, however, malignant cell spreading and seeding is of big concern. Since approximately 20–50 % of all underlying causes are malignant, explorative surgery is favored in adults.     

Carcinosarcoma of the liver: report of a case

A 64-year-old Japanese woman without a history of viral hepatitis was admitted for investigation of a huge liver mass. The tumor, measuring 14 × 12 × 22 cm, had invaded the diaphragm, right lung, and inferior vena cava. Serum examinations demonstrated high levels of carbohydrate antigen 19-9 (CA19-9), and the Child-Pugh score was A. She underwent right lobectomy of the liver and partial resection of the right diaphragm, right lung, and inferior vena cava. Radio- and chemotherapy were also given, but she died of recurrence 3 months after surgery. Microscopically, the tumor exhibited intermingled adenocarcinomatous and atypical mesenchymal components. The carcinomatous component was positive for cytokeratins 7, 19, and 20, chromogranin A, epithelial membrane antigen, c-KIT, and vimentin. The sarcomatous component was positive for vimentin and c-KIT. A review of 36 cases of hepatic carcinosarcoma revealed the following: chronic hepatitis or cirrhosis in 57 % of the patients; increased serum CA19-9 levels in 30 %; a mean tumor diameter of 10 cm; invasion of the adjacent organs or metastasis to distant organs in 47 %; wide intrahepatic infiltration in 44 %; and 50 % survival of only 5 months. Significant differences were seen according to tumor diameter (diameter >5 cm; p < 0.05), wide intrahepatic infiltration (p < 0.05), and extrahepatic invasion/metastasis (p < 0.01). Neither chemotherapy nor radiotherapy contributed to prognosis, but surgical resection resulted in some improvement (p < 0.05).     

Riesiges intrathorakales benignes Teratom

Mature teratoma are rare, usually benign germ cell tumors. We report a case of a 41-year-old woman who received a chest X-ray due to inspiratory sharp left thoracic pain with flu-like infection. The x-ray showed an unclear cardiac dilatation. After echocardiographic clarification, a chest CT was performed. This showed a left thoracic, polycystic tumor (11?×?13?×?17?cm). The tumor could be resected in toto. The histological examination revealed a mature cystic teratoma. Due to the risk of rupture, infiltration or malignancy each thoracic teratoma should be radically resected surgically.     

Ectopic cervico-mediastinal thymoma confirmed by flow cytometric analysis of tumor-derived lymphocytes

Ectopic cervical or cervico-mediastinal thymomas are very rare and most of them are asymptomatic, except for the presence of a cervical mass. We present the case of a 71-year-old man with an ectopic cervico-mediastinal thymoma threatening superior vena cava syndrome. He had a slight headache and presented with venous dilatation on the chest wall. A computed tomographic scan and magnetic resonance, imaging of the chest demonstrated a mass extending from the right neck to the hilum, that indented the trachea and compressed and displaced the brachiocephalic veins anteriorly. Under a right hemicollar incision and median sternotomy, the mass was resected en bloc together with the thymus. The resected specimen was an encapsulated mass measuring 11×7×4 cm. The pathological diagnosis was type AB, non-invasive thymoma, confirmed by 3-color flow, cytometry of tumor-derived lymphocytes. Flow cytometry using biopsy material may contribute to the preoperative diagnosis of ectopic thymoma.     

Giant cell tumors that originated in the sternum

Giant cell tumors originating from the sternum are rare. We report a case of a giant cell tumor of the sternum with radiological evidence of aggressiveness. A 34 year-old woman noted a mass in the anterior chest wall that had been slowly growing over 1 year. After incision biopsy revealed a diagnosis of a giant cell tumor she was treated by surgical resection (subtotal sternectomy) and reconstruction with methylmethacrylate. The tumor was 14 × 9 × 8 cm, and histological study confirmed that it was a giant cell tumor. Although giant cell tumors are benign, they are locally aggressive lesions and must be considered in the differential diagnosis in patients with a sternal mass.     

A rapidly growing benign intrathoracic neurofibroma after lung lobectomy.

A 67-year-old male underwent a right upper lung lobectomy for lung cancer in January 1993. Follow-up chest X-rays revealed a progressive and rapidly growing intrathoracic mass in the right thorax. The mass, however, did not resemble a tumor recurrence, and the patient complained only of shortness of breath. Computerized tomography and magnetic resonance imaging confirmed the presence of the intrathoracic mass and its associated compression of the residual lung. A right thoracotomy was performed in January 1998, and a mass found arising from the sympathetic nerve trunk was resected. Microscopic examination revealed stellate or spindle-shaped cells in myxoid stroma with sparsely distributed collagen fibers. Immunohistochemically, the cells were positive for neuron-specific enolase, and the tumor was identified as neurofibroma. The patient did not suffer from von Recklinghausen's disease, and there was no family history of the disease. After resection of the neurofibroma, the compressed lung was able to re-expand, and the patient's shortness of breath disappeared. At one year postoperative, the patient remains well, and there is no evidence of recurrence.