Humans ; Paraganglioma/complications* ; Retroperitoneal Neoplasms/diagnostic imaging* ; Retroperitoneal Space ; Tachycardia

Klinefelter syndrome (KS) is often associated with various neoplasms, especially germ cell tumors. Mediastinum is the most favored site of extragonadal germ cell tumors with KS, which is somewhat different from those without KS. The retroperitoneal germ cell tumor in KS is very rare. A five-month-old boy with an abdominal mass was found to have a retroperitoneal tumor. After surgical removal, he was diagnosed to have mature cystic teratoma. Cytogenetic study of his peripheral lymphocytes revealed that his karyotype was consistent with KS. This case suggests that patients with KS might be at risk of having germ cell tumors in sites other than mediastinum. It also suggests that all cases with these tumors should be screened for the presence of karyotypic abnormalities, and it might help to assess the exact correlation between germ cell tumors and KS, and to treat them accordingly.
Case Report ; Human ; Infant ; Karyotyping ; Klinefelter's Syndrome/genetics ; Klinefelter's Syndrome/complications* ; Male ; Retroperitoneal Neoplasms/pathology ; Retroperitoneal Neoplasms/complications* ; Teratoma/pathology ; Teratoma/etiology*

Adult ; Giant Cell Tumor of Bone ; complications ; Humans ; Male ; Retroperitoneal Neoplasms ; secondary

To improve the diagnosis and treatment of pulmonary lymphangiomyomatosis, clinical data for the first successfully treated case of pulmonary and retroperitoneal lymphangiomyomatosis in our hospital has been comprehensively analyzed, and the relevant literature has been reviewed. A 45-year-old Han Chinese woman initially presented six months ago with increasing shortness of breath on exertion and was admitted to our hospital after four days of chest pain. Admission examination revealed chylothorax, interstitial lung disease, and enlarged retroperitoneal lymph nodes. The patient was finally diagnosed with pulmonary and retroperitoneal lymphangiomyomatosis based on laparotomy examination and biopsy of the retroperitoneal lymph nodes. After six months of rapamycin treatment, the symptoms - lung function, arterial blood gas, and imaging of the patient- were improved significantly. Pulmonary lymphangiomyomatosis clinically manifests as progressive dyspnea, recurrent pneumothorax, and chylothorax, and can be diagnosed by its characteristic features in high-resolution computed tomographic images or pathological examination. The successful treatment of pulmonary lymphangiomyomatosis with rapamycin brings new hope to those afflicted with this disease.
Female ; Humans ; Lung Neoplasms ; complications ; drug therapy ; Lymphangioleiomyomatosis ; drug therapy ; Middle Aged ; Retroperitoneal Neoplasms ; complications ; drug therapy ; Sirolimus ; therapeutic use

Acute Kidney Injury ; diagnostic imaging ; etiology ; Aged ; Humans ; Male ; Necrosis ; diagnostic imaging ; etiology ; Paraganglioma ; complications ; Postoperative Complications ; Retroperitoneal Neoplasms ; complications ; Skin ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

Carney triad is a rare syndrome of unknown etiology characterized by having at least two out of three following neoplasms: gastrointestinal stromal tumor, pulmonary chondroma and extra-adrenal paraganglioma. About 100 cases have been reported worldwide. We report a case of Carney triad in a 42-year-old woman presented with a gastrointestinal stromal tumor in the stomach and a malignant functioning paraganglioma in the retroperitoneum that was fatal five years after diagnosis. The gastrointestinal stromal tumor was diagnosed as intermediate-risk of aggressive behavior and diffusely positive for c-kit whereas the retroperitoneal paraganglioma was negative for c-kit. Genetic analyses showed no mutations of KIT, PDGFRA, SDHB, SDHC, and SDHD genes in both tumors. To our best knowledge, this is the first case of Carney triad in Korea.
Adult ; Carney Complex/*chemically induced/complications ; Female ; Gastrointestinal Stromal Tumors/*diagnosis/pathology ; Humans ; Korea ; Paraganglioma/*diagnosis/pathology ; Retroperitoneal Neoplasms/*diagnosis/pathology ; Stomach Neoplasms/*diagnosis/pathology

Haemangioma of the retroperitoneal space is a rare benign capillary malformation, which can grow significantly in pregnancy due to the multiple associated cardiovascular changes. We herein describe the case of a pregnant woman with an extensive right retroperitoneal haemangioma extending from the level of the renal hilum, across the lateral anterior abdominal wall and into the thigh. We also highlight the challenges faced in the management of the patient's delivery process. To the best of our knowledge, this is the first case of such nature and severity described in the English literature.
Adult ; Cesarean Section ; Female ; Gestational Age ; Hemangioma ; diagnosis ; surgery ; Humans ; Magnetic Resonance Imaging ; Pregnancy ; Pregnancy Complications, Neoplastic ; Retroperitoneal Neoplasms ; diagnosis ; surgery

OBJECTIVETo compare and evaluate the clinical value among transperitoneal, retroperitoneal laparoscopic adrenalectomy and open adrenalectomy for the treatment of adrenal tumours.

METHODSFrom January 1996 to June 2002, transperitoneal laparoscopic adrenalectomy (TLA) was performed for 19 cases, 17 cases were successful (Group A), retroperitoneal laparoscopic adrenalectomy (RLA) for 21 cases, 19 cases were successful (Group B), during the same period 41 patients with adrenal tumours under 6 cm in diameter underwent open adrenalectomy (Group C). The procedure was evaluated and compared in the respects of tumors weight, operation time, blood loss, recovery time of GI function, hospital stay, complications transferable rate and indications among three groups.

RESULTSAverage tumors weight was (13.86) g in group A, (15.66) g in group B and (18.03) g in group C; mean operating time: group A was 17.21 min longer than group B, group A. B was 48.53 min longer and 31.32 min longer han group C respectively, mean blood loss in group A. B was less diminished by 121.55 ml and 137.05 ml than group C, and the blood loss in group B was less diminished by 15.50 ml than group A; mean recovery of Gl function, group A and B was 1.87 day and 2.17 day earlier than group C respectively, group B was 0.3 day earlier than group A, mean hospital stay was 2.60 day and 3.87 day shorter than group C, group B was 1.27 day shorter, than group A; transferable rate of operation was 10.5% (group A) and 9.5% (group B). Postoperative complication rate was 10.5%, 14.3% and 9.7% in group A. B and C respectively.

CONCLUSIONSTLA and RLA were better than open method. On the hand of approach to adrenal gland disturbs to abdominal organ and postoperative recovery on RLA have some slight advantage.

Adrenal Gland Neoplasms ; surgery ; Adrenalectomy ; methods ; Adult ; Aged ; Female ; Humans ; Laparoscopy ; methods ; Laparotomy ; Male ; Middle Aged ; Postoperative Complications ; prevention & control ; Retroperitoneal Space ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo search the etiologic factor, clinical diagnosis points and treatment of postoperative intussusception (PI).

METHODSTo retrospectively review the clinical materials of 14 cases with PI including the cause of disease and treatment.

RESULTSPI occurred within 10 days (average 4 days) after the primary operation. Bowel obstructive symptoms gradually emerged. One case was diagnosed with intussusception by sonography and received enema reduction of intussusception by hydrostatic pressure. Thirteen cases were performed secondary operation. Small intestine was main site of intussusception. Manual reduction of the lesion was performed in 12 cases and bowel resection and anastomosis was done in 1 case with bowel necrosis.

CONCLUSIONPI should be suspected if child presents with the symptoms of ileus in early postoperative period. Abdominal sonography may have some value on diagnosis of PI. Operation is the first choice for the treatment of PI.

Child ; Child, Preschool ; Female ; Humans ; Ileal Diseases ; diagnosis ; etiology ; surgery ; Infant ; Intestine, Small ; surgery ; Intussusception ; diagnosis ; etiology ; surgery ; Male ; Postoperative Complications ; Retroperitoneal Neoplasms ; surgery ; Retrospective Studies ; Teratoma ; surgery

INTRODUCTIONBladder cancer is a common malignancy but presentation with metastatic disease is rare. This is the fi rst reported case of duodenal obstruction as a presentation of metastatic bladder cancer.

CLINICAL PICTUREA middle-aged woman presented with nausea, vomiting, weight loss and intermittent haematuria. Radiology and histology confirmed metastatic bladder cancer to the retroperitoneum encasing the duodenum and causing obstruction.

TREATMENTInsertion of a duodenal stent relieved the obstruction and palliative chemoradiotherapy was initiated.

OUTCOMEThe patient died 15 months after diagnosis.

CONCLUSIONSClinicians and radiologists should be aware of atypical presentations of common malignancies.

Adult ; Carcinoma, Transitional Cell ; drug therapy ; secondary ; Diagnosis, Differential ; Duodenal Obstruction ; diagnosis ; etiology ; surgery ; Fatal Outcome ; Female ; Humans ; Palliative Care ; Retroperitoneal Neoplasms ; complications ; diagnosis ; secondary ; Stents ; Urinary Bladder Neoplasms ; drug therapy ; pathology