Unusual metastasis of renal carcinoma

Renal carcinoma, the third most common urological cancer, induces presence of metastases in 75% of cases. The most affected sites for metastasis are the lungs, the lymphatic system, bones, the liver, adrenal glands and the brain with sometimes a cancer free period of several years prior to evolutionary recurrence of the disease. The aim of this literature review is to report on secondary uncommon renal localizations by underlining their clinical significance, as well as main characteristics, in order to provide guidelines for effective patient diagnosis and therapeutic management.     

Malignant hereditary paraganglioma: problems raised by non-functional forms management

Non-functional paraganglioma have not clinical or biological characteristics, so that the diagnostic is most of the time delayed and made on the occasion of advanced abdominal tumor or symptomatic metastasis management. Hereditary forms, notably those with SDHB mutation, seem to have a poor prognosis. On the other hand, and on the oposite to sporadic forms, they are the only ones to benefit from genetic testing which make possible, if positive, an earlier diagnostic, before apparition of symptoms, recurrence or metastasis. We report a case of non-functional malignant hereditary paraganglioma diagnosed belatedly and we will consider management problems raised by non-functional forms.     

Gangliocytic paraganglioma of the duodenum.

In spite of possessing microscopic features of invasiveness, the clinical behaviour of duodenal gangliocytic paraganglioma is benign. The operation of choice for removal of the tumour is local excision. This conservative approach was used in the case of a 70-year-old white women who presented with occult gastrointestinal bleeding. The unusual histologic features of this woman's tumour are discussed. Although the tumour appears to be aggressive no recurrence has been reported to date following adequate local excision of a duodenal gangliocytic paraganglioma.     

Gangliocytic paraganglioma of the ampulla of Vater

A case of a bleeding duodenal gangliocytic paraganglioma (GPG) of the Papilla Vateri is reported. The 47 year old patient was treated successfully by partial pancreaticoduodenectomy. Histologically the GPG is characterized by three types of cells: epitheloid cells, ganglion-like cells and spindle cells. So far 81 cases are reported in the literature. Most cases of GPGs are benign, metastasis or recurrence is possible. This case showed an infiltrative growth pattern indicating the true neoplastic nature of this tumor.     

Laparoscopic pancreatic resections

The feasibility of laparoscopic pancreatic resection has been demonstrated. However, the real clinical benefit for the patients remains questioned. The best indication for a laparoscopic approach appears to be the resection of benign or neuro-endocrine tumors without a need for pancreato-enteric reconstruction (i.e enucleation or distal pancreatectomy). The use of the laparoscopic approach for malignant tumors still remains controversial. The benefits of minimally invasive surgery are clearly correlated with the successful management of the pancreatic stump. Pancreatic related complication rate (fistula and collection) is 15% when using pancreatic transection with a laparoscopic endostappler.     

Surgical treatment of pancreatic metastases from renal carcinoma

OBJECTIVES: Pancreatic metastases from renal carcinoma are poorly known. The aim of this study was to report clinical and radiological manifestations, the treatment performed, and the observed survival in 7 patients with this rare entity. RESULTS: All patients were operated on. One patient had nonresectable tumor. Six patients underwent curative resection. There was one postoperative death. Follow-up after pancreatectomy ranged from 6 months to 3 years. Two patients developed extra-pancreatic metastases one year and 3 years after pancreatectomy respectively. CONCLUSIONS: Pancreatic metastases from renal carcinoma are rare and often occur several years after nephrectomy. However their resection is often possible and allows a good long-term survival. PATIENTS AND METHODS: From 1988 to 2000, 7 patients (5 men and 2 women, mean age = 66 years) with pancreatic metastases from a renal cell carcinoma were observed in the same center. One patient had synchronous metastasis; in the 6 others, metastases were diagnosed 4 to 16 years after nephrectomy, and were revealed by pain (n = 2), gastrointestinal bleeding (n = 1), faintness (n = 1) or routine follow-up (n = 2). The diagnosis of metastases was made by contrast-enhanced abdominal CT-scan.     

Intraductal papillary mucinous tumor of the pancreas degenerated and fistulisated in the stomach, the duodenum and colon

We report a case of intraductal papillary mucinous tumor of the pancreas (IPMT) degenerated and fistulized into the stomach, the duodenum and the colon. Diagnoses of malignancy and fistulization were proven preoperatively. Pancreaticoduodenectomy extended to the body was performed. Pathological examination revealed a malignant IPMT with an invasive component. Of the 3 fistulizations tracts, both gastric and duodenal ones were malignant. To our knowledge, this is the first reported case of IPMT with colonic fistulization. Twelve months later, the patient is alive without signs of recurrence.     

Evaluation of a method to reduce the number of sentinel nodes removed in melanoma patients: prospective study

INTRODUCTION: Since 1992, sentinel node (SLN) biopsy was generally applied to melanoma for carcinologic staging. Literature points out an increase of nodes removed for each procedure. It means to a high cost for this procedure and it wanders from the defining concept of SLN. The aim of our study was to evaluate whether, we can minimize number of SLN removed, without influencing the reliability of carcinologic staging. MATERIAL AND METHODS: We conducted a prospective study about 50 patients with stage one melanoma. For each patient, the SLN was identified with hand-held gamma probe technique. We removed only the hottest and all nodes greater than 70% of the hottest. We analysed the characteristics of melanoma, the success rate of this procedure, how many nodes have been removed and how many have had micro-metastases. This result was compared to two main studies with chi(2) test. RESULTS: The success rate of this technique was 100%. We dissected 1,3 SLN for each patient, with 22% positive SLN. Statistical analyse pointed out a better selectivity of our study, rate of pathological positivity and recurrence was alike. DISCUSSION: Our technique decreasing number of removed SLN is reliable. A minimal number of nodes doesn't distort sensitivity of carcinologic staging, and reduce cost of the procedure.     

Major hepatectomy for metastasis of colorectal cancer improves survival in the elderly

OBJECTIVE: Assessing impact of major liver resection (LR) for hepatic metastasis of colorectal cancer (HMCC) on post operative courses and long term survival in the elderly. PATIENTS AND METHOD: Thirty-three consecutive patients aged over 70 years-old were treated in our institution for up to 3 resectable metachronous HMCC. Fifteen patients had major LR (9 right hepatectomy, 3 extended right hepatectomy, 3 left hepatectomy) without pre or postoperative chemotherapy (group 1) and 18 patients were exclusively treated by chemotherapy (group 2) because of high ASA score (ASA 3) or patients refusal. RESULTS: No patients died of another cause that colorectal cancer disease during observation time. All patients of group 2 died during observation time. Post operative mortality and morbidity of group 1 were respectively 0% and 33%. Survival at 1 and 2 years of group 1-2 were respectively 73-50% (P=0,04) and 47-15% (P=0,05). Median survival of group 1 and 2 were respectively 22 and 12 months (P=0,03). CONCLUSIONS: Major LR for HMCC could be proposed regardless the age. High ASA score, multiple (more than 4) metastasis location, evolutive disease could justify an exclusive medical approach.     

Gangliocytic paraganglioma of the bronchus: a case report with follow-up and ultrastructural assessment

We report a case of gangliocytic paraganglioma of bronchus. A 54-year-old woman underwent bronchoscopy following two episodes of right lower lobe pneumonia over the previous 5 months with unresolved chest radiographic changes. A computerized tomographic scan showed a right lower lobe endobronchial lesion, and at bronchoscopy there was a mass partly occluding the lumen of the bronchus. The biopsy and subsequent bronchoscopic resection showed a tumor with morphologic, immunohistochemical, and ultrastructural features of paragangliomatous, gangliocytic, and Schwann cell differentiation consistent with a gangliocytic paraganglioma. The lesion was treated conservatively with bronchoscopic resection and laser therapy. Histopathologic examination of recurrent tumor at 6 months showed features consistent with paraganglioma. Ten months after initial diagnosis, there was no bronchoscopic evidence of residual tumor. The occurrence of gangliocytic paraganglioma in diverse sites gives cause for the reappraisal of the histogenesis of this fascinating lesion. The variable morphology of this lesion may be an expression of the potential for divergent differentiation of a pluripotent stem cell.     

Surgical management of a preaortic paraganglioma: Report of one case

Paragangliomas (PG) are rare and often diagnosed in the young adult. One case of retroperitoneal preaortic paraganglioma localised between the celiac trunk and the superior mesenteric artery is reported. The management of paraganglioma involves endocrinologists, geneticists and surgeons but the only potentially curative treatment remains surgical resection. Pathology reports can not always discriminate between benign or malignant tumors. Hereditary in paraganglioma occurs in approximately 25% of cases. Genetic investigation is therefore mandatory in all patients with PG. Since the type of genetic mutation is correlated with tumoral aggressiveness, genetic investigation results should be taken into account when a surgical procedure is planned.     

Arterial reconstruction with detubulated aortic patch in simultaneous kidney-pancreas transplantation

Simultaneous pancreas kidney transplantation has become an accepted therapy for the treatment of patients with insulino-dependant diabetes and renal chronic failure. The arterial arrangement of the pancreatic graft is necessary in order to avoid surgical complications of vascular thrombosis. We reported three cases of simultaneous pancreas kidney, a simple procedure using aortic arterial patch preleved with the superior mesenteric artery and detubulated, than the splenic artery is directly anastomosed to the patch.     

Lymphoepithelial cyst of the pancreas. A case report

Lymphoepithelial cyst of the pancreas is a benign and rare pathology. Its histogenesis is still unknown. The diagnosis is difficult to establish before surgery. We report a new case of a 20-year-old woman admitted for abdominal pain and vomiting. Radiologic investigations described a multilocular cystic tumor of the tail of the pancreas. The patient underwent a left pancreatectomy with splenectomy. Histologic investigations revealed pancreatic cysts lined by squamous epithelium surrounded by dense lymphoid tissue. The diagnosis of lymphoepithelial cyst of the pancreas was done.     

Bilateral Leydig cell tumor of the test: a case report

Testicular Leydig cell tumours are uncommon. Bilateral synchronous lesions are exceptional. They cause isosexual pseudo precocious puberty in childhood. The histological diagnosis of malignancy is sometimes difficult to establish and it can be made retrospectively when lymph nodes involvement or visceral metastasis appear in the follow-up. We report a case of a 9 year-old boy presenting bilateral Leydig cell tumour of the testis treated by bilateral radical orchiectomy who developed 2 years after the intervention a pulmonary metastasis.     

Results of resection for ductal adenocarcinoma of the pancreatic head

INTRODUCTION: Pancreaticoduodenectomy (PD) is the only curative treatment for adenocarcinoma of the pancreatic head but is associated with a significant early morbidity and a poor long term survival. Therefore, its value is still debated. The aim of this study was to evaluate early and distant results of PD for pancreatic adenocarcinoma, and to identify prognostic factors. SUMMARY: Seventy-nine patients who underwent PD with curative intent for adenocarcinoma of the pancreatic head from 1982 to 2002 were studied retrospectively. The following data were evaluated: operative mortality, long-term survival, prognostic factors (through univariate and multivariate analysis), and characteristics of 5-year survivors. RESULTS: Mortality rate was 1.3%. Survival at 1, 3 and 5 years was 46%, 26% and 11%. The median survival was 12 months. The prognostic factors were the T stage (T.N.M. classification) and radicality of resection. After multivariate analysis, radicality of resection was the only independent prognostic factor. Five patients survived for more than 5 years. They did not differ of the other patients but none had positive margin or venous invasion. CONCLUSIONS: These results (low mortality, significant distant survival including some long term survivors) suggest that PD for pancreatic adenocarcinoma must be indicated in most low-risk patients. PD remains the only curative treatment allowing prolonged survival.     

Hyperostotic paraganglioma of occipitotemporal bone

A 25-year-old male presented with off-and-on vertigo of 10-year duration. He had left-sided cerebellar signs, left vocal cord paresis, and minimal left-sided hearing impairment. Computed tomography of the head revealed significant hyperostosis of squamous occipital, mastoid, and petrous temporal bone with no adjacent soft tissue mass. On excision, it turned out to be paraganglioma. Paraganglioma as a pure bony mass is not reported in the literature. The site of origin of such a tumor could not be ascertained, even on generous screening. The tumor remained nonsecretory on clinical and biochemical investigations.     

Radiosurgery for paraganglioma of the temporal bone

PURPOSE: To report the outcome of patients with paraganglioma of the temporal bone treated with stereotactic radiosurgery at the University of Florida. METHODS AND MATERIALS: Between January 1997 and June 1999, five patients with paraganglioma of the temporal bone were treated with Linac-based stereotactic radiosurgery at the University of Florida. The ages of the three female and two male patients were between 40 and 88 years (median, 49 years). Four patients were treated at initial presentation, and one had recurrent disease. Treatment volumes ranged from 4.9 cm3 to 18.4 cm3, with a mean of 10.84 cm3. The dose applied to the margin of the tumor varied from 12.5 to 15 Gy (median, 15 Gy). The treatment dose was specified to the 80% isodose shell in two cases and to the 70% isodose shell in three cases. The median follow-up time was 27 months, ranging from 14 to 50 months. RESULTS: One of four previously untreated patients had a relapse at the primary tumor site. Treatment failure occurred at the field margin 6 months after radiosurgery; the patient was subsequently treated with fractionated stereotactic radiotherapy and at the time of analysis had no evidence of disease, 21 months after initiation of salvage therapy. The patient treated at the time of recurrence after conventional radiotherapy had a local recurrence 40 months after radiosurgery. At the time of this recurrence, the patient had biopsy-proven metastatic disease in two cervical lymph nodes, and no salvage therapy was performed. All patients were alive at the time of the analysis, one with disease present. Presenting symptoms improved in two patients and stabilized in one. The two patients who had local recurrence develop had worsening of their symptoms. One patient had a cranial nerve V palsy develop 6 months after treatment, which resolved after a few months. CONCLUSIONS: In this series, the results with stereotactic radiosurgery are discouraging compared with our results with conventional fractionated radiotherapy in patients with paraganglioma of the temporal bone.     

Spontaneous subcapsular hematoma of the kidney. Report of 6 cases

Spontaneous subcapsular haematoma (SCH) of the kidney is a rare condition, secondary to kidney tumors in more than 50% of cases. Diagnosis is suggested by sonography and confirmed by CT-scan. Renal angiography, performed in haemodynamically stable patients, shows the origin of bleeding and allows embolization. The authors report 6 cases of SCH of the kidney, one of which occurred after extra-corporeal shock-wave lithotripsy.