[Parathyroid carcinoma: clinical case and review of the literature]. / Carcinoma paratiroideo: caso clinico e revisione della letteratura.

INTRODUCTION: Parathyroid carcinoma is a rare endocrine neoplasm, difficult to define clinically and histopathologically. CASE REPORT: The case concerns of a 20 years old male with situs viscerum inversus (with dextrocardia), and symptoms: asthenia, oliguresis, nausea, emesis, myalgia, lower limb paresthesia and very high levels of calcium and PTH. Laboratory findings (PTH 580 pg/ml; Ca 12.40 mg/dl; P 1.9 mg/dl), echography, TC, and parathyroid scintigraphy, associated with clinical data, have suggested hypothesis of parathyroid carcinoma confirmed by histological examination and immunochemistry. Surgery was efficacious with normalization of Calcium and PTH levels, and disappearance of symptoms. After two years no signs of local recurrence were present, but imaging show pulmonary micronodulations of uncertain pathological meaning. DISCUSSION AND CONCLUSION: No clinical or bio-humoral data allows a preoperative diagnosis of parathyroid carcinoma. Only with definitive pathology and immunohistochemistry it is possible to differentiate an adenoma from a carcinoma. Surgery is the only effective therapy and therefore should be always performed. This neoplasm usually relapses, locally first and later with distant metastases. For this reason after surgery the patients should always undergo a strict follow-up programme including evaluation of PTH and calcemia.

Peritoneal mesothelioma in a case of inguinal hernia. A review of the literature.

The peritoneal mesothelioma (PM) is a rare, benign or malignant, primary tumour, arising from the peritoneal membrane. The most frequent histological form is the malignant one with an incidence of 2-2.6 new cases per million per year. The symptomatology is insidious and poses difficult problems in the diagnosis and the treatment. Instrumental diagnostic investigations are useful only in the diagnostic orientation. Only the pathologic examination allows to distinguish a peritoneal carcinomatosis from PM. The prognosis of MPM is pour. An intense multimodal therapy, combining surgery with CT and RT, increases the survival rates in the patients with MPM. It has been proposed that hernia of abdominal wall play a role in the pathogenesis of this tumor. We believe that hypothesis seems unlikely considering the enormous discrepancy between the incidence of hernial pathology and PM.

Lateral pelvic lymphadenectomy and total nerve sparing for locally advanced rectal cancer in Western patients.

BACKGROUND: In an attempt to improve the results of locally advanced rectal cancer treatment, we performed an extended surgical technique consisting of total mesorectal excision (TME), lateral pelvic lymphadenectomy (LPL) and total nerve sparing (NS). Resection of the autonomic nerves was realized only when these fibres were involved by the tumour. METHODS: Nine cases (9.2%) of a personal series of 98 western patients with rectal carcinoma operated on between January 1992 and December 1997 at Third Department of Surgery, University La Sapienza, Rome, underwent TME, LPL and NS procedures for locally advanced extraperitoneal disease. RESULTS: Two out of seven patients in stage II/III suffered postoperatively from urinary retention with mild irregular flow as tested on urodynamics, but no long-term urinary disturbances persisted. Retrograde ejaculation occurred postoperatively in one of two patients who experienced urinary disturbances. Another patient had erection dysfunctions. These sexual dysfunctions did not improve during the long-term follow-up. Seven patients with stage II or stage III disease achieved a 5-year survival rate of 80.0% and a 5-year disease-free survival rate of 68.6% after a mean follow-up period of 64.7 months. None of them experienced local recurrence, but one patient died of diffuse metastatic disease 50 months after surgery. Two patients with stage IV rectal carcinoma died of local and distant disease 13 months and 35 months after operation. One patient underwent liver resection for solitary metastasis 25 months after primary operation. CONCLUSIONS: TME, LPL, and NS with resection of autonomic nerves only when these fibres are involved by the disease can achieve satisfactory results in terms of survival and functional outcome in selected western patients with locally advanced rectal cancer.

[Compatibility, indications, and limits of nerve sparing technique in lateral pelvic lymphadenectomy for advanced rectal carcinoma]. / Compatibilità, indicazioni e limiti della nerve sparing technique nella linfadenectomia pelvica laterale per carcinoma del retto avanzato.

The aim of radical surgical treatment of rectal cancer is to control the spread and prevent recurrence of the disease. In an attempt to improve the results of treatment of locally advanced rectal cancer, we advocate an extended surgical approach consisting of total mesorectal excision, lateral pelvic lymphadenectomy and the nerve sparing technique with resection of autonomic nerves whenever these fibers are affected by locally advanced tumor. Nine cases (9.2%) in a personal series of 98 patients with rectal carcinoma, operated on over the period from January 1992 to December 1997, underwent total mesorectal excision, lateral pelvic lymphadenectomy and the nerve sparing technique procedures for locally advanced extraperitoneal disease. In 7 patients with stage II or III disease, the 5-year survival rate was 80% and the 5-year disease-free survival rate 66.7% after a mean follow-up of 55 months. None of them experienced local recurrence, but one patient died of diffuse metastatic disease 50 months after surgery. One patient with stage IV rectal cancer died of disease 13 months postoperatively, while another patient with the same stage of disease is still alive with disease 26 months after surgery. One patient underwent liver resection for a solitary metastasis 25 months after the primary operation. Two patients suffered postoperatively from urinary retention with mild irregular flow at urodynamic testing, but no long-term urinary disturbances persisted. Retrograde ejaculation occurred postoperatively in one of the two patients who experienced urinary disorders, and another patient had erection disturbances. These sexual dysfunctions did not improve during long-term follow-up. Total mesorectal excision, lateral pelvic lymphadenectomy, and the nerve sparing technique, with resection of the autonomic nerves whenever these fibers are involved, allow satisfactory results to be achieved in terms of survival and functional outcome in patients with locally advanced rectal cancer. In western subjects, however, this procedure is safe only after careful patient selection.

Evaluation of anal function after surgery for rectal cancer.

BACKGROUND AND OBJECTIVES: Sphincter-saving surgical procedures for rectal cancer have been legitimized if executed respecting the criteria of oncological radicalness. Our objective was to evaluate anal sphincteric continence after rectal cancer surgery. METHODS: A detailed questionnaire regarding continence was administered to 3 groups of patients. Group 1 was composed of 9 patients treated with a higher (>4 cm), stapled colorectal anastomosis; the 9 group 2 patients were treated with a low (< or =4 cm), stapled colorectal anastomosis; the 9 group 3 patients underwent coloanal anastomosis. RESULTS: The results were evaluated about 3 years after surgery. Continence was excellent in group 1 and very good in group 2. However, in group 3, we observed diminished gas/feces discrimination, reduced ability to postpone evacuation, and increased soiling and perianal rash. CONCLUSIONS: Anal sphincteric continence was better after surgery with a high or low colorectal anastomosis than after coloanal anastomosis.

Local recurrences and primary surgery in rectal carcinoma.

BACKGROUND: The authors analyze retrospectively a consecutive series of rectal carcinomas operated on with different surgical strategies at Third Surgical Department of "La Sapienza" University of Rome, between January 1985 and December 1997, by one expert surgeon (GDM), and report the incidence of the local recurrence correlated to the surgical technique development. METHODS: In most recent groups of patients treated for extraperitoneal neoplasm from January 1992 with curative (R0) total mesorectal excision (TME) and nerve sparing technique (NST) (Group C, n = 47) and with curative TME plus lateral pelvic lymphadenectomy (LPL) and NST (Group D, n = 7), sacrificing the pelvic autonomic nervous system only in case of neoplastic infiltration, the local recurrence was 8.5% (4 cases, with mean interval of 30.5 months) and 0 respectively at mean follow-up of 44.9 and 55 months. RESULTS: In none of the local recurrences of the Group C a re-resection (neither curative nor palliative) was possible and the survival was 50% at 14 months from the diagnosis of relapse. Instead, in local recurrences of rectal carcinoma in patients who underwent a first anterior resection with less extended dissection in other Department (Group E), a re-resection was possible for 3 cases out of 4 (R0, R1 and R2 operations); re-resected patients are now alive at mean follow-up of 33.6 months (82, 12 and 7 months, respectively). In Group A patients, treated between January 1985 and December 1988 with partial mesorectal excision (R0) also for extraperitoneal localization, the incidence of local relapses is 21.9% (9/41 cases) vs 11.2% (11/98 cases) in Group B patients, treated from January 1989 with curative TME for extraperitoneal tumors. CONCLUSIONS: The incidence of local recurrences of extraperitoneal rectal cancer can be reduced by total mesorectal excision. The total sparing of pelvic autonomic nervous system in advanced rectal carcinoma doesn't increase the incidence of local recurrences.

Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: report of a case.

Tuberous sclerosis is a hereditary autosomal-dominant disease characterized by hamartomas that can develop in any organ. We report herein the case of a 34-year-old female with tuberous sclerosis and a huge abdominopelvic mass that started growing quickly 2 years after its diagnosis. The patient had undergone several previous operations for hydrocephalus and cerebral tubers, and a nephrectomy for right renal angiomyolipoma. On admission, she was in poor general health with renal failure, severe anemia, and weight loss. A laparotomy revealed that the tumor occupied the pelvis, the lower and part of the upper abdomen, and was hypervascularized, with an extremely irregular surface covered in nodules, vegetations, and areas of hemorrhagic necrosis. The development of the mass and the impossibility of recognizing the internal genital organs led us to assume that the formation had originated from these. Frozen-section examination indicated an undifferentiated tumor that had not been completely resected. Her postoperative course was complicated by bronchopneumonia and progressive renal failure. The patient died 10 days after surgery due to cardiorespiratory failure. A histological diagnosis of epithelioid angiomyolipoma was confirmed. Although it is presently impossible to determine whether angiomyolipoma with predominant epithelioid cells is more aggressive than typical angiomyolipoma, it definitively demonstrated local aggressive behavior in this patient.

[Traditional cholecystectomy in a patient with situs viscerum inversus partialis]. / Colecistectomia per via tradizionale in paziente con situs viscerum inversus partialis.

The authors discuss the etiology of situs viscerum inversus partialis (SVIP), the associated anomalies and the malformations and the relative clinical problems, reviewing the international literature. The present a case of a 51 years old female with a diagnosis of umbilical hernia and cystocele. During hospitalization the patient complained a typical acute cholecystitis pain. The patient underwent ultrasonography, CT scan, and MNR that allowed us to diagnosis a calculous cholecystitis with SVIP and was elected for an open cholecystectomy. The Authors describe the surgical technique in relation to the anatomic anomalies, in particular vascular ones, that were discovered with the imaging studies and confirmed at laparotomy.

[Our experience in the recurrence of rectal cancer operated with radical intention in light of a case load treated in a homogeneous manner]. / La nostra esperienza delle recidive di cancri rettali operati con intento radicale alla luce dei risultati di una casistica trattata in maniera omogenea.

A reappraisal of the results obtained after potentially curative resection for rectal cancer compared with "historical" results is reported. An increase of the overall survival rates was registered as well as a corresponding lowering of the pelvic recurrences: 4.54% in the group of patients with a-two-year follow up. As for relapse surgery, however, reviewing the series from January 1991 to December 1994, the results are still poor because relapses were not resectable in 85.7% of the cases.