Lymphoproliferative disorders presenting as mediastinal neoplasms

Lymphoproliferative disorders may present in any organ of the body. The mediastinum is an uncommon location for presentation of these heterogeneous disorders, but involvement of the mediastinum may be the sole site of disease for several aggressive lymphomas. Both Hodgkin's disease and non-Hodgkin's lymphoma may present in the mediastinum. The most common types of non-Hodgkin's lymphoma involving the mediastinum include lymphoblastic lymphoma and mediastinal large cell lymphoma. These lymphomas most commonly develop in the anterior mediastinum but may be seen in the middle and posterior mediastinum. Symptoms associated with a mediastinal presentation of a lymphoproliferative disorder are often attributable to compression of mediastinal structures (eg, superior vena cava syndrome) or invasion of thoracic structures such as the pericardium or pleura. Although staging can be performed with routine imaging studies, surgical intervention is often required to ensure accurate histologic diagnosis of these lymphomas. Once a diagnosis has been established, therapeutic modalities usually include chemotherapy and/or radiotherapy.     

Mediastinal masses in children

A variety of mediastinal masses may present in children. Imaging is generally confined to chest radiograph and CAT scan of the chest, although other studies may prove helpful depending upon the circumstances. MRI is particularly helpful for neurogenic tumors in the posterior mediastinum. The most common tumor in the anterior mediastinum is lymphoma with germ cell tumors second. In general, lymph nodes in areas outside the mediastinum provide access for tissue diagnosis when lymphoma is present. Germ cell tumors are generally benign, but surgical excision is indicated to rule out malignant elements and to treat symptoms which are often present through compression of nearby structures. Neurogenic tumors are the most common lesions in the posterior mediastinum.     

Malignant Peripheral Nerve Sheath Tumor Arising from the Left Ventricle

Abstract We report a case of a 32-year-old female who underwent complete excision of a malignant left ventricular peripheral nerve sheath tumor. Malignant peripheral nerve sheath tumors in the mediastinum, including the pericardium, are rare. This case report describes a malignant peripheral nerve sheath tumor arising from the left ventricular origin for which complete excision was possible. (J Card Surg 2012;27:567-570).     

Surgery for thymoma.

Although thymoma is an uncommon tumor, it represents the most frequently encountered tumor of the anterior mediastinum. These tumors represent an interesting and even peculiar group of lesions by virtue of their association with paraneoplastic disorders, their relatively indolent course, and their predisposition for local recurrence. The initial treatment of choice for patients with thymoma that do not present with unresectable local or diffuse metastatic disease is complete surgical resection. The goals of surgery are complete excision of the lesion with total thymectomy and complete exploration to rule out the presence of noncontiguous disease that may be resectable. Often, complete resection may require the resection of surrounding involved structures including pericardium, pleura, lung, and even major vascular structures. Some authors have suggested VATS or VATS-assisted techniques for small thymomas. Capsular invasion, however, often can be subtle, and the completeness of resection is of prime importance in countless studies. With recurrences appearing up to 5 and even 10 years postoperatively, time will tell if these minimally invasive techniques are comparable with current standard approaches. Multiple studies have failed to determine conclusively the role of induction chemotherapy and adjuvant radiation. Prospective multi-institutional trials are required to elucidate further the role of such therapies in these rare tumors. In the interim, the authors continue to recommend postoperative radiation for all patients undergoing resection with the exception of stage I patients. Some promising reports on response to chemotherapy have led them to develop an induction chemotherapy protocol for patients with clinically advanced disease.     

Bronchogenic cysts above and below the diaphragm: report of eight cases

Bronchogenic cysts are developmental foregut anomalies usually located within the mediastinum. Although typically asymptomatic, infection or compression of adjacent structures may become prominent. Surgical excision is recommended to establish diagnosis, alleviate symptoms if present, and prevent future complications. We report our recent experience with bronchogenic cysts including 7 located within the mediastinum and a rare instance of a cyst below the diaphragm.     

Successful Resection of a Recurrent Mediastinal Liposarcoma Invading the Pericardium: Report of a Case

Primary liposarcoma of the mediastinum is rare, but cases of recurrence have been reported in the English literature. We successfully resected a recurrent pericardial liposarcoma, detected 5 years after the initial resection of a liposarcoma of the anterior mediastinum invading the pericardium. Routine follow-up computed tomography showed the recurrence and suggested invasion of the pericardial cavity, which was supported by the findings of transesophageal ultrasonography. As cine-magnetic resonance imaging suggested that the tumor was resectable, an operation was performed. Histopathology confirmed the diagnosis of recurrent liposarcoma and showed clear surgical margins.     

Application of pericardial graft in the surgical management of Peyronie's disease

PURPOSE: We investigated the use of Tutoplast cadaveric pericardium as an alternative material for grafting the tunica albuginea defect after Peyronie's plaque excision. MATERIALS AND METHODS: A total of 11 patients with significant penile curvature interfering with sexual intercourse were evaluated after at least 12 months of conservative therapy. Preoperative evaluation in all cases included penile duplex Doppler ultrasound studies. Chemically processed and gamma irradiated pericardium was used to graft the cavernosal defect after surgical excision of the penile plaque. In 3 patients a penile prosthesis was simultaneously placed secondary to documented erectile dysfunction at duplex Doppler ultrasound evaluation. RESULTS: Penile curvature resolved in all patients, allowing for normal sexual function after a mean followup of 14 months (range 9 to 19). No postoperative evidence of tissue rejection or infection was noted. In 1 patient urethro-corporal communication developed and a single penile cylinder was explanted. Another patient had a mild dorsal bulge at the site of graft placement when the prosthesis was fully inflated. CONCLUSIONS: Polymeric silicone material used to provide satisfactory results in the surgical treatment of Peyronie's disease but it is no longer available for biomedical use. We demonstrate the effective use of cadaveric pericardium as a graft material for cavernosal defects after Peyronie's plaque excision. Overall patient satisfaction, absence of a second surgical incision, relative low risk and ease of surgical placement make this material ideal for the surgical treatment of this disease process.     

Retroperitoneal Castleman’s disease mimicking soft tissue tumour

Castleman’s disease is a type of non-neoplastic lymphoproliferative disease having lymph nodal hyperplasia. It has two distinct microscopic types: hyaline-vascular type and plasma cell type. Clinically, it may present either as a solitary mass, most commonly in the mediastinum, or as a multicentric form whose features are generalized lymph-adenopathy, splenomegaly and involvement of other organs like the lungs and kidneys. Here we report a case of isolated retroperitoneal Castleman’s disease, which presented as a lump in the iliac fossa in a young female. A clinico-radio-logical diagnosis of retroperitoneal soft tissue tumour was made and the patient underwent complete surgical excision. The exact diagnosis was only obtained at histopathology and there is no evidence of recurrence at six months follow-up.     

Peripheral primitive neuroectodermal tumor of the thymus

Peripheral primitive neuroectodermal tumors (pPNET) are an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usually occur in children and adolescents with a predilection for the truncal and axial soft tissue. Thoracopulmonary manifestation in children mostly involves the chest wall as a large tumorous mass or the posterior mediastinum. In adults these tumors are even more infrequent. We report about a 46-year-old woman with a pPNET of the thymus which infiltrated the pericardium. According to the English literature this is the first reported case both of a thymic pPNET and a pPNET arising in the anterior mediastinum. This illustrates that pPNETs belong to the differential diagnosis of neurogenic mediastinal malignancies not only in the pediatric age population but also in adults and even in so far unknown localizations like the anterior mediastinum.     

Mediastinal hibernoma.

A 46-year-old asymptomatic male was detected to have a posterior mediastinal mass on a routine check-up. He underwent thoracotomy to remove the mass, which was found to be a hibernoma. The mediastinum is an extremely rare site for an even rarer tumor like the hibernoma. An additional unique feature was the very large tumor size despite which the patient was asymptomatic. Imaging studies are not helpful in revealing its clinically indeterminate nature, hence a surgical specimen is necessary to establish the correct diagnosis. Total excision is advocated for cure, as there is no known malignant potential.     

Primary mesothelioma of the pericardium

A case of primary mesothelioma of the pericardium is presented. The diagnosis was established after surgical excision. A brief review of the literature is included.     

Life-threatening giant mediastinal cystic teratoma in a 4-month-old male baby

Cystic teratomas of the mediastinum are rare and present with severe respiratory distress during childhood. Aspiration of the cyst is indicated to reduce the cyst volume, alleviate the patient’s symptoms, and secure an operative field. This report describes a 4-month-old male baby with a large anterior mediastinal teratoma presenting with severe respiratory distress. The cystic teratoma was large enough to cause pectus carinatum. Respiratory distress secondary to airway obstruction was markedly reduced by percutaneous aspiration of the cyst. Surgical excision is indicated for a malignant or benign well-demarcated mass in any part of the mediastinum. In our patient, total surgical excision was performed. Preoperatively, percutaneous aspiration was performed twice, with the symptoms being reduced after each aspiration. Surgical excision is the best means of diagnosing and treating a benign teratoma. Percutaneous cyst aspiration not only improved ventilation but also facilitated easy excision of the cyst during surgical management.     

Unicentric Castleman's disease in peripancreatic tissue: case report and review of the literature

Castleman's disease is a rare disorder of the lymphoid tissue that usually occurs in the mediastinum even if extrathoracic involvement, including neck, axilla, mesentery and retroperitoneum, has also been described. We report a case of a 69 years old men with peripancreatic localisation, mimicking a pancreatic neoplasm treated with local excision. Only seven cases of pancreatic and peripancreatic localisation are described in the world-wide literature. This particular site of disease may give troublesome differential diagnosis due to non specific clinical signs and radiological features. Often surgical excision is both diagnostic and therapeutic.     

Extrapulmonary intrathoracic hydatid cysts.

INTRODUCTION: Hydatid disease, a clinical entity endemic in many sheep- and cattle-raising areas, is still an important health problem in the world. Extrapulmonary location of cysts in the thorax is rare. We report our experience with intrathoracic but extrapulmonary hydatid cysts and discuss concepts of treatment. METHOD: In our Thoracic and Cardiovascular Surgery Department at the Dicle University School of Medicine, 133 patients with thoracic hydatid cysts were managed surgically between January 1990 and October 2002. In 14 (10.5%), the cysts were extrapulmonary but within the thorax, located in the pleural cavity, mediastinum, pericardium and diaphragm, or in pleural fissures. Cysts were intact in 12 patients and ruptured in 2. Radiographs of the chest were the main means of diagnosis; all patients with mediastinal and diaphragmatic cysts and some with pleural cysts were also scanned with computed tomography. All patients were managed surgically. RESULTS: We operated on 3 mediastinal, 2 diapragmatic and 1 pericardial hydatid cyst, as well as 6 in pleural fissures and 2 in the pleural space. Lateral thoracotomy was chosen as the surgical incision in all patients except 1 (7% of the 14), who had median sternotomy for a pericardial hydatid cyst. Empyema developed in 2 patients (morbidity, 14%). No patient died perioperatively. CONCLUSIONS: Hydatid cysts may be found in many different sites. Surgery to obtain a complete cure is the treatment of choice for most patients with intrathoracic but extrapulmonary cysts; excision must be done without delay to avoid or relieve compression of surrounding vital structures.     

Compound Odontoma

A case of compound odontomas of the anterior maxilla is discussed. Multiple tooth like structures were discovered upon surgical excision.     

Versatility of a mini-trapdoor incision in upper mediastinal exposure

Exposure of the upper mediastinum and thoracic outlet can pose major surgical challenges. We report our application of a previously described mini-trapdoor incision to a variety of surgical problems involving the upper mediastinum and thoracic outlet, including subclavian vein thrombosis, penetrating subclavian artery injury, debridement of subjacent chest wall infection, lymph node excision, and Pancoast tumor resection. This versatile approach provides excellent structural visualization while obviating clavicular resection or sternoclavicular joint disruption, or both.     

Conservative management of cervicomediastinal chyloma in an infant

The accumulation of chyle into the mediastinum after surgical intervention is considered an extremely rare event, which has been previously described only in adulthood. We report on a 9-month-old boy who developed a cervicomediastinal chyloma after excision of a large left-sided supraclavicular cystic hygroma. Notwithstanding considerable tracheal deviation, the infant remained asymptomatic and was successfully treated by nonoperative treatment. Professionals should be aware of such an unusual condition, which may benefit from expectant management when evaluation establishes a high likelihood of clinical diagnosis, and patient's general condition remains stable. This approach may avoid, with little risk, unnecessary early surgical intervention in some of these patients.     

Primary mediastinal leiomyosarcoma

Primary leiomyosarcoma of the mediastinum is a very rare malignant mesenchymal tumor, which may arise in the soft tissue of the mediastinum or from the great vessels. Due to its rarity and nonspecific clinical symptoms, the correct diagnosis and proper management are often delayed and not well established. We report a 55-year-old woman with a large right-sided mediastinal leiomyosarcoma with adhesion to the pericardium and right middle lobe, which was completely removed via right thoracotomy along with pericardial resection and right middle lobectomy.     

Supradiaphragmatic renal cell carcinoma tumor thrombus: indications for vena caval reconstruction with pericardium

Supradiaphragmatic extension of tumor thrombus from a renal cell carcinoma presents a major surgical challenge. The use of cardiopulmonary bypass, hypothermia and cardiac arrest with temporary exsanguination has allowed for successful surgical excision of these tumors. A renal cell carcinoma on the right side with a supradiaphragmatic tumor thrombus still may only partially occlude the vena cava. The collateral venous circulation of the left renal vein may be developed poorly and a pericardial patch can allow successful reconstruction of the inferior vena cava. If the tumor arises on the left side continued venous drainage of the right kidney is mandatory to prevent venous infarction of the right kidney. The entire vena cava also might be reconstructed theoretically with pericardium.