Periportal Plexiform Neurofibromatosis; a rare intra-abdominal manifestation of NF1.

Periportal Plexiform Neurofibromatosis (PPNF) is a rare visceral manifestation of Neurofibromatosis Type 1 (NF1) or Von Recklinghausen disease. Neurofibromas are the most common lesions in NF1. We present a case of a young female diagnosed with NF1 who initially presented with hard abdominal mass. Contrast enhanced CT revealed the unusual lintrahepatic periportal plexiform neurofibromatosis in addition to a typical large retroperitoneal lumbar neurofibroma.

Intrapelvic Retroperitoneal Synovial Sarcoma in a 15-Year-Old Adolescent Girl: A Case Report and Review of the Literature.

Synovial sarcomas are a rare subtype of soft tissue sarcomas mostly located in the lower extremities. The authors report a case of synovial sarcoma in a 15-year-old adolescent girl with several unusual features including age, intrapelvic retroperitoneal location of the primary tumor, and presentation with right abdominal tenderness and compression of the iliac vessels with thrombosis of the right iliac and femoral vein.

Effect of nephrectomy for retroperitoneal sarcoma on post-operative renal function.

BACKGROUND AND OBJECTIVES: Surgical resection of retroperitoneal sarcomas (RPS) often involves removal of the ipsilateral kidney. We evaluated the long-term post-operative renal function in patients who underwent a nephrectomy as part of their en-bloc resection of RPS. METHODS: Retrospective review of an institutional database identified RPS patients who underwent nephrectomy for curative intent from 1990 to 2014. The primary outcome measured was chronic kidney disease (CKD) calculated by the glomerular filtration rate (GFR). RESULTS: Of the 47 patients in our study, 19 (40%), 18 (38%), and 10 (21%) patients had a preoperative CKD stage 1, 2, and 3, respectively. The GFR decreased by an average of 33.4 mL/min/1.73 m2 with 66% of patients demonstrating mild progression of their renal impairment. Only three (6%) patients progressed to CKD stage 4 or 5, one of which required life-long dialysis. CONCLUSION: Nephrectomy as part of an en-bloc resection is associated with a decrease in GFR that is not clinically significant. Fear of kidney failure should not prevent a surgeon from performing a nephrectomy in the treatment of RPS.

A paraganglioma in a hypertensive patient with unilateral renal hypoplasia.

We report the case of a 46-year-old hypertensive Japanese female with renal insufficiency related to unilateral renal hypoplasia. The patient was found to have developed paraganglioma in the retroperitoneal space over a 5-year period. Catecholamine-producing tumors are not usually recognized as conditions associated with renal hypoplasia. Our long-term observation of the patient eventually led us to the diagnosis of paraganglioma. In hypertensive patients with chronic kidney disease, not only the renin-angiotensin-aldosterone system but also catecholamine activity may be involved, particularly in the patients whose cases are complicated with unilateral renal hypoplasia.

Long-term follow-up results of the observation program for neuroblastoma detected at 6-month mass screening.

We conducted an observation program of neuroblastoma in infants, detected by mass screening at 6 months of age; we followed up with them for 15 years. No recurrence was observed after disappearance of tumors, and persistent tumors showed no malignant transformation or metastasis. Histology of the resected tumors showed age-related differentiation.

Cyclic fluctuation of blood pressure in neonatal neuroblastoma.

Herein is described a case of neonatal neuroblastoma with cyclic blood pressure fluctuation and elevated catecholamines. The fluctuations stabilized after treatment with α-adrenergic blocker and the perioperative course was uneventful. The possibility of catecholamine-related symptoms including hypertension, heart failure, and blood pressure fluctuations should be considered in the treatment for neuroblastoma; if they are present, treatment with α-blockers is effective.

[A case of incidentally diagnosed retroperitoneal lymphangioleiomyomatosis with no respiratory symptoms].

A 39-year-old woman presented with a large retroperitoneal tumor found incidentally in a routine examination. The 138×37×26 mm mass was located in the left paraaortic region. Blood tests and urinalyses including endocrinological examinations revealed no abnormalities. A chest computed tomography revealed multiple thin-walled pulmonary cysts, which is a characteristic of lymphangioleiomyomatosis (LAM). Because the findings strongly suggested that the retroperitoneal tumor was an extrapulmonary manifestion of LAM, we performed laparoscopic resection of the tumor for diagnosis and treatment. The pathological diagnosis was LAM. The tumor cells were immunohistochemically positive for α -smooth muscle actin and weakly positive for HMB45, which is consistent with LAM. The cells were also positive for estrogen receptor (ER) and progesterone receptor (PgR). LAM is a rare progressive disease that affects mainly the lung, and leads to chronic respiratory failure. Extrapulmonary LAM without respiratory symptoms, is extremely rare. In the past, the prognosis of LAM was poor, with a median survival of 8-10 years, but now 85% survive more than 10 years. In the present case, deterioration of pulmonary lesions was not observed during the 10 months follow-up. Because ERand PgRfindings were positive, we will consider hormonal therapy as a treatment option, when the pulmonary lesions progress in the present case.

[An autopsy case of retroperitoneal paraganglioma that had presented with pheochromocytoma multisystem crisis].

A 40's woman was seen at the emergency room because of severe back pain and vomiting. Abdominal CT scan revealed a tumor about 7cm in diameter located on the retroperitoneum. For 6 hours after admission, her body temperature was 39°C and respiratory failure occurred. After 30 hours, the patient died because of a complication in circulatory failure. From the abnormally high level of catecholamines in the blood and autopsy results, we determined that pheochromocytoma multisystem crisis had been caused by the retroperitoneal paraganglioma.

Cross-femoral venous by-pass (Palma's procedure) to relieve venous hypertension due to retroperitoneal leiomyosarcoma: a case report.

Retroperitoneal leiomyosarcoma arising in, or involving, the inferior vena cava or the iliac vein are infrequent tumours. They are usually treated by "en-block" surgery. Venous reconstruction is usually not needed, since collateral circulation can adequately offset the main vessel flow. However, in rare cases, collateral circulation may be insufficient. The authors report a case of leiomyosarcoma infiltrating the left iliac vein. One week after radical resection surgery, the patient developed left leg compartmental syndrome due to venous hypertension. The patient was successfully treated by thrombectomy of the left femoral vein and cross-femoral venous bypass (Palma's Procedure) by means of an 8 mm diameter polytetrafluoroethylene (PTFE) prosthesis. To improve the flow inside the graft, an arterio-venous fistula (AVF) was performed. The choice of a prosthetic conduit, its diameter, and the AVF are discussed.

[Invasive tumor pain therapy in the final stage of a cervix cancer patient]. / Invasive Tumorschmerztherapie bei terminalem Verlauf einer Zervixkarzinom-Patientin.

Cancer disease is associated with a lot of very burdening symptoms. Pain is with dyspnea and emesis in the first time in a rate of about 50%, in the terminal stage up to 90% the most tormenting symptom. For more than 90% of patients the 3-step-WHO-regimen for cancer pain is able to relieve pain successfully. But a little group of cancer patients need invasive measures and methods to relieve pain sufficiently. This would be the 4th step, according to the enlarged WHO-regimen. Intravenous or intrathecal applications, eventually regional blockades should be considered. The following case-report shows the steps of a difficult cancer pain therapy in a regional general hospital. It is sometimes not evidence based, on the one hand because of necessary organizational, staff-related, or other makeshifts in daily clinical practice, on the other hand because of the lack of appropriate EBM-guidelines. The pressure to reduce the pain effectively and as simple as possible produces sometimes polypragmatic, retrospective not always satisfying measures.

The Role of Tc-99m DTPA Renal Dynamic Scintigraphy in Retroperitoneal Liposarcoma.

PURPOSE: Technetium-99m diethylene triamine pentaacetic acid (Tc-99m DTPA) renal dynamic scintigraphy is a widely used imaging technique that evaluates renal function of patients with extrarenal abnormalities, but its clinical value in potentially offering us information on proliferation of liposarcoma has not yet been reported. METHODS: We retrospectively reviewed 7 patients with histopathologically confirmed retroperitoneal liposarcoma who underwent Tc-99m DTPA renal dynamic scintigraphy. The clinical data, histopathological findings, Glomerular Filtration Rate (GFR), and Tc-99m DTPA uptake were recorded. RESULTS: Dedifferentiated liposarcoma and well-differentiated liposarcoma showed dissimilar degrees of Tc-99m DTPA uptake, an observation that correlated with Ki-67 expression (p < 0.01). 4 of the 7 patients were diagnosed with dedifferentiated liposarcoma, showing a moderate uptake of Tc-99m DTPA and greater than 20% Ki-67 expression on histological slides. Meanwhile, the remaining 3 patients, diagnosed with well-differentiated liposarcoma, showed no uptake of Tc-99m DTPA and Ki-67 expression of less than 5%. CONCLUSIONS: This study suggests that Tc-99m DTPA renal dynamic scintigraphy provides diagnostic value in patients with retroperitoneal liposarcoma, not only in evaluating renal function but also in visualizing lesion-related radionuclide uptake, which may potentially offer further clinical insights into tumor proliferation and provide prognostic value for clinical outcomes in patients with retroperitoneal liposarcoma.

Ifosfamide encephalopathy and use of methylene blue. A case report of different sequential neurotoxicity.

Neurotoxicity is a clinically relevant adverse event observed with the use of ifosfamide. It is usually mild, occasionally severe and seldom fatal. Ifosfamide-induced encephalopathy requires interruption of chemotherapy, intravenous hydration and administration of methylene blue. Less is known about the efficacy of methylene blue in avoiding a second episode of ifosfamide-induced encephalopathy while maintaining chemotherapy with ifosfamide. We report a case of a different clinical manifestation of ifosfamide-induced encephalopathy after continued ifosfamide use and despite methylene blue in a patient with retroperitoneal sarcoma.

The natural history of incidental retroperitoneal schwannomas.

The natural history of asymptomatic retroperitoneal schwannomas is poorly understood. This study aimed at investigating the natural history of incidental retroperitoneal schwannomas. The medical charts and imaging studies of 22 asymptomatic patients under observation for at least 12 months for retroperitoneal schwannomas were reviewed. The duration of follow-up ranged between 13 and 176 months (mean 48 months). In the 22 patients managed by the "wait and see" approach, the average tumor size at initial presentation was 51 mm, which increased to 57 mm at final follow-up. During the final follow-up, 2 patients required surgical treatment for tumor enlargement, while the remaining patients remained asymptomatic without surgery. The average growth rate of the tumors was 1.9 mm/year (range: -1.9 to 8.7 mm/year). The majority of asymptomatic retroperitoneal schwannomas demonstrate minimal growth and may be suitable for management with the "wait and see" approach.

Surgical management of retroperitoneal schwannoma complicated with severe hydronephrosis: A case report.

RATIONALE: Schwannomas are usually benign tumors arising from well-differentiated schwann cells, which rarely occur in the retroperitoneal space. The lack of specific signs and radiologic imaging characteristics makes preoperative diagnosis rather difficult. Most retroperitoneal schwannomas are benign and the primary treatment choice for retroperitoneal schwannomas is surgical excision, however, the involvement of the urinary system is scarcely reported. PATIENT CONCERNS: A 34-year-old woman presented with progressive left abdominal pain and rebound abdominal mass at the left lower quadrant for 1 month. Radiological imaging suggested capsulated solid mass with cystic and necrotic areas in the retroperitoneum accompanied by severe left kidney hydronephrosis and preoperative biopsy result was inconclusive. DIAGNOSES: We believe this is a rare case of retroperitoneal schwannoma complicated with severe hydronephrosis. INTERVENTIONS: After preparation, the patient underwent laparoscopy exploration and converted to open surgical exploration. The patient accepted complete surgical excision of the retroperitoneal tumor and left kidney. Postoperative pathology diagnosis of the mass was proven to be benign retroperitoneal schwannoma. OUTCOMES: Postoperative course of the patient was uneventful and the left abdominal pain was greatly improved. After 12-month follow up, no evidence of recurrence or any other complication including renal failure was observed. LESSONS: Preoperative imaging and preoperative ultrasound-guided biopsy are helpful to make accurate diagnosis. The final diagnosis is based on postoperative histological and immunohistochemical findings. The primary treatment option is complete surgical resection of the retroperitoneal schwannoma and the involved upper urinary system when severe hydronephrosis occured. Local recurrence and overall survival are closely correlated with negative resection margins and pathology types.

Retroperitoneal ganglioneuroma with lymphnode metastasis: a case report.

Ganglioneuroma is a benign, most differentiated tumor of neural crest origin. These tumors though benign, can very rarely metastasize to regional lymphnodes or to distant sites. We report a case of a 52 year old lady who presented to the surgical OPD with dyspeptic symptoms of 2 months duration. Ultrasonography and CT scan of the abdomen showed a homogenous mass in the region of the right adrenal gland, extending between the inferior vena cava and the aorta. A clinical and radiological diagnosis of an adrenal tumor was made. Microscopically, the tumor histology was that of a ganglioneuroma (schwannian stroma-dominant) mature type. The lymph node attached to the tumor showed metastasis, the metastatic foci having the same morphology as the primary tumor. The right adrenal gland was free of tumor. The case is illustrative due to its clinical presentation mimicking an adrenal incidentaloma, and a rare phenomenon of a benign tumor exhibiting completely differentiated lymph node metastasis.