OBJECTIVETo investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB).

METHODFrom May 2011 to December 2013, 6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital, Peking University. The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago. A retrospective analysis for the location, stage, pathological type, treatment way and outcome of neuroblastoma was done.

RESULT(1) All patients were misdiagnosed as simply opsoclonus-myoclonus syndrome (OMS) at the time of onset. They had been receiving treatment with adrenocorticotropic hormone and intravenous immunoglobulin within 1-13 months.OMS-NB was diagnosed by means of enhanced abdominal CT image which was delayed to be given after the poor efficacy or relapse. (2) The primary tumors were almost all small, stage I-II, located in adrenal, retroperitoneal or pelvis. The pathology of tumors included ganglioneuroblastoma (5/6) and neuroblastoma (1/6). (3) All these cases underwent surgery, 4/6 cases with complete tumor resection, 2/6 cases with tumor around the aorta and induced local residue. Preoperative and postoperative chemotherapy was given to 2 and 5 cases, respectively. (4) The patients were followed up for 3-31 months, except 1 patient lost, the other 5 are currently surviving disease-free (3 having been at the end of chemotherapy, 1 still in chemotherapy, and another had local recurrence and is receiving radiotherapy and chemotherapy after the second operation and now also stopped taking the medicine). The symptoms of nervous system have been significantly improved during postoperative chemotherapy.

CONCLUSIONTo reduce the misdiagnosis, regular CT imaging of the abdomen or pelvic should be ordered for all cases with OMS. The children with OMS-NB need to be actively treated with the combined modality therapy including surgery, chemotherapy or radiotherapy, to reduce recurrence and reduce the symptoms of nervous system.

Adrenal Gland Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Antineoplastic Agents ; administration & dosage ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Biomarkers ; analysis ; Chemotherapy, Adjuvant ; Child, Preschool ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Neoplasm Recurrence, Local ; surgery ; therapy ; Neoplasm Staging ; Neuroblastoma ; complications ; diagnosis ; surgery ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; etiology ; therapy ; Retroperitoneal Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

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

Primary retroperitoneal mucinous cystadenoma is an extremely uncommon tumor, even though mucinous cystadenoma often develops in the ovary and less frequently in the pancreas. A 21-year-old female was admitted to our hospital due to severe abdominal pain. A well-demarcated, oval shaped cystic tumor at the retropancreatic area with displacement of the pancreas and surrounding major vessels was observed on CT and MRI. Exploratory laparotomy was performed, and complete excision of the entire cyst was performed without complication. The pathologic finding was consistent with primary retropancreatic mucinous cystadenoma. To the best of our knowledge, this report is the first to describe a case of retropancreatic mucinous cystadenoma arising from the retropancreatic area in Korea.
Antibodies/metabolism ; Cystadenoma, Mucinous/*diagnosis/pathology/surgery ; Female ; Humans ; Magnetic Resonance Imaging ; Mucin 5AC/immunology ; Mucin-2/immunology ; Ovarian Neoplasms/*diagnosis/pathology/surgery ; Retroperitoneal Neoplasms/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo compare the safety, feasibility and efficacy of transperitoneal and retroperitoneal laparoscopic partial nephrectomy (LPN) in the treatment of renal tumors with R. E. N. A. L score more than 7.

METHODSThe clinical data were collected from 62 patients undergoing transperitoneal LPN (32 cases) and retroperitoneal LPN (30 cases) for a complex renal mass (R.E.N.A.L. score≥7) between January 2012 and March 2014. The surgical and early postoperative outcomes and complications were analyzed to evaluate the efficacy of the treatments. The mean operative time, estimated blood loss, warm ischemia time, surgical complications, blood transfusion rate, tolerating regular diet time, postoperative hospital stay and surgical margin were compared between the two groups.

RESULTSThe operations were completed successfully in all cases except for 1 case in transperitoneal group and 3 in retroperitoneal group that required conversion to open surgery. No significant differences were found in age, body mass index, ASA score, Charlson comorbidity index, tumor size or R.E.N.A.L. nephrometry score (P>0.05), nor in estimated blood loss, warm ischemia time, intraoperative complication, blood transfusion rate or surgical margin between the two groups (P>0.05, respectively). The transperitoneal LPN group had a shorter mean operative time than retroperitoneal LPN group (210.4∓59.2 vs 252∓58.3 min, P<0.05) but showed longer tolerating regular diet time (47∓10 h vs 23∓6 h, P<0.05) and postoperative hospital stay time (8.4∓1.9 days vs 6.5∓1.6 days, P<0.05).

CONCLUSIONBoth transperitoneal LPN and retroperitoneal LPN are safe, feasible and effective for surgical management of complex localized tumors, but the transperitoneal procedure offers larger operative space with better exposure; the retroperitoneal procedure better promotes postoperative recovery of the patients.

Humans ; Kidney Neoplasms ; diagnosis ; surgery ; Laparoscopy ; Length of Stay ; Nephrectomy ; Operative Time ; Retroperitoneal Space ; Retrospective Studies ; Treatment Outcome

Adult ; Humans ; Male ; Pulmonary Embolism ; diagnosis ; surgery ; Retroperitoneal Neoplasms ; diagnosis ; surgery

Abdominal Neoplasms ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Liposarcoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Retroperitoneal Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

Resection of retroperitoneal tumors is usually perfomed using the anterior retroperitoneal approach. Our report presents an innovative method utilizing a robotic surgical system. A 50-yr-old male patient visited our hospital due to a known paravertebral mass. Magnetic resonance imaging showed a well-encapsulated mass slightly abutting the abdominal aorta and left psoas muscle at the L4-L5 level. The tumor seemed to be originated from the prevertebral sympathetic plexus or lumbosacral trunk and contained traversing vessels around the tumor capsule. A full-time robotic transperitoneal tumor resection was performed. Three trocars were used for the robotic camera and working arms. The da Vinci Surgical System(R) provided delicate dissection in the small space and the tumor was completely removed without damage to the surrounding organs and great vessels. This case demonstrates the feasibility of robotic resection in retroperitoneal space. Robotic surgery offered less invasiveness in contrast to conventional open surgery.
Aorta, Abdominal ; Humans ; Image Processing, Computer-Assisted/instrumentation ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neurilemmoma/diagnosis/*surgery ; Retroperitoneal Space ; Robotics/*instrumentation ; Spinal Neoplasms/diagnosis/*surgery ; Surgery, Computer-Assisted/*instrumentation

Schwannoma is a benign neoplasm of the Schwann cells of the neural sheath. Most schwannomas occur in the head and neck, and extremities and rarely in the retroperitoneal space. The differentiation of a schwannoma from other malignant tumor or benign tumor is very difficult on a preoperative examination with ultrasonography, computed tomography or magnetic resonance imaging. Furthermore, the lesion with increased fluorodeoxyglucose uptake in PET-CT cannot exclude malignant tumor. Therefore, this lesion needs surgical excision and a histological examination with immunohistochemical staining. We report a case of schwannoma occuring in the retroperitoneal space that incidentally discovered by PET-CT for health-check up. Pathologic confirmation by laparoscopic excision was done.
Aged ; Diagnosis, Differential ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Male ; Neurilemmoma/*diagnosis/pathology/surgery ; Positron-Emission Tomography ; Retroperitoneal Neoplasms/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed

Solitary fibrous tumors (SFTs) are an uncommon neoplasm characterized by the proliferation of spindle cells. The diagnostic criteria of malignant solitary fibrous tumors (MSFTs) include high cellularity, high mitotic activity (4>10 HPF), pleomorphism, hemorrhage and necrosis. This tumor frequently involves the pleura and MSFTs of retroperitoneum mimicking gastric submucosal tumor are very rare. We report a rare case of MSFT that presented as a gastric submucosal tumor. A gastroscopic examination showed a large bulging mucosa in the gastric body. Abdominal computed tomography revealed a well-defined heterogeneous enhancing mass between the left hepatic lobe and gastric body. Surgical resection was performed and histologic features were consistent with a MSFT.
Antigens, CD34/metabolism ; Gastroscopy ; Humans ; Male ; Middle Aged ; Proto-Oncogene Proteins c-bcl-2/metabolism ; Retroperitoneal Neoplasms/*diagnosis/pathology/surgery ; Solitary Fibrous Tumors/*diagnosis/pathology/surgery ; Stomach Neoplasms/diagnosis ; Tomography, X-Ray Computed

OBJECTIVEThe purpose of this study was to assess the imaging characteristics of abdominal pheochromocytoma in multiphasic spiral CT scanning, and to determine whether these image characteristics can aid in differentiating pheochromocytoma from other types of tumors or not.

METHODSThe image data of dynamic enhanced CT of 79 pathologically confirmed pheochromocytomas in 70 patients were retrospectively reviewed.

RESULTSAmong the 70 patients, there were 41 patients with endocrine symptoms related to pheochromocytoma, 15 had a latent pheochromocytoma and the remaining 14 cases presented with a non-functioning pheochromocytoma. There were totally 79 tumors in 70 patients, with a single lesion in 63 cases, while multiple lesions in the other 7. Sixty cases had a tumor located in the adrenal gland, while 8 in retroperitoneal space, and 2 cases had both intraadrenal and ectopic lesions simultaneously. Sixty patients had a benign pheochromocytoma, the other 10 had a malignant one or relapse after operation. The average size of the tumor was 5.8 cm (range, 2 approximately 15 cm in the longest diameter). Seventy-seven pheochromocytomas had a well defined boundary except two big ones with a unclear margin, which were finally proven to be malignant. Homogeneous enhancement was found in 6 lesions with a diameter

CONCLUSIONApproximately half of the abdominal pheochromocytomas are lack of endocrine symptoms related with their tumors. However, they may display some typical CT characteristics, such as that a small lesion is often homogeneous but hypervascular, a larger tumor may present hemorrhage, necrosis, and cystic change with rich or moderate blood supply. However, when a small tumor has moderate blood supply, it should be differentiated with an adrenal adenoma; when a big one has moderate blood supply, it should be differentiated with other malignant tumors. Furthermore, a part of malignant pheochromocytomas is really difficult to be differentiated from some benign lesions by spiral CT images alone.

Adenoma ; blood supply ; diagnostic imaging ; surgery ; Adolescent ; Adrenal Gland Neoplasms ; blood supply ; diagnostic imaging ; surgery ; Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Pheochromocytoma ; blood supply ; diagnostic imaging ; surgery ; Radiographic Image Enhancement ; Retroperitoneal Neoplasms ; blood supply ; diagnostic imaging ; surgery ; Retrospective Studies ; Tomography, Spiral Computed ; methods ; Young Adult