Jonathan A. van Heerden

Long-term evaluation following resection of apparently benign pheochromocytoma(s)/paraganglioma(s).

AbstractIn the absence of distant disease, the pathologic diagnosis of malignancy in pheochromocytoma or paraganglioma is impossible. In an effort to establish the true incidence of recurrence in this disease, we have analyzed long-term follow-up (average, 15.8 years) of 98 patients who underwent complete resection of localized, noninvasive, histologically-benign pheochromocytomas and paragangliomas at our institution between 1960 and 1976.nEighty-eight patients had nonfamilial, sporadic pheochromoctyoma/ paraganglioma. Nine had multiple endocrine neoplasia (MEN) type 2 (2A: 7, 2B: 2), and 1 had familial pheochromocytoma. Seventy-nine patients had single pheochromocytomas; 10 had single extraadrenal tumors (paragangliomas); and 9 had multicentric or bilateral adrenal tumors.nSix patients (6.5%) developed recurrent pheochromocytoma after documentation of normal postoperative urinary catecholamine levels. One of these patients had MEN 2A. The recurrences developed at intervals from 5 to 13 years following initial resection. These were distant in 3 patients, local in 2, and both local and distant in a single patient. None of the recurrences occurred in the 13 patients who, on pathologic rereview, had either local or vascular invasion. No paraganglioma recurred.nLife-long follow-up of all patients who have had pheochromocytomas or paragangliomas resected is mandatory.RésuméEn labsence de lésions pathologiques à distance, le diagnostic de malignité en matière de phéochromocytome ou de paraganglion est impossible. Pour déterminer la fréquence vraie de récidive nous avons analysé le suivi à long terme (moyenne, 15.8 ans) de 98 patients ayant eu une résection complète de phéochromocytome et de paraganglions bénins localisés et non invasifs opérés à la Clinique Mayo entre 1960 et 1976.Quatre-vingt-huit patients avaient un phéochromocytome ou paraganglion sporadique, non familial. Neuf patients avaient une MEN de type 2 (2A: 7, 2B: 2), et un patient avait un phéochromocytome familial. Soixante-neuf patients avaient un phéochromocytome simple; 10 avaient une tumeur extrasurrénalienne simple (paraganglion); et 9 avaient des tumeurs surrénales bilatérales ou multicentriques.Six patients (6.5%) ont eu une récidive de leur phéochromocytome après normalisation des catécholamines urinaires en postopératoire. Un de ces patients avait une MEN 2A. La récidive sest manifestée entre 5 et 13 ans après la résection. Elle était à distance chez 3 patients, locale chez 2 patients, et à la fois locale et à distance chez un. Il ny avait aucune récidive chez les 13 patients qui avaient soit une invasion locale ou vasculaire lorsque les coupes histologiques ont été revues. Il ny avait aucune récidive de paraganglion.La surveillance permanente de tout patient ayant un phéochromocytome ou un paraganglion semble simposer.ResumenEn ausencia de metástasis distantes, el diagnóstico histopatológico de malignidad en un feocromocitoma o en un paraganglioma es imposible. Con el objeto de establecer la verdadera incidencia de recurrencia de esta enfermedad, hemos analizado el seguimiento a largo plazo (promedio, 15.8 años) de 98 pacientes sometidos a resección completa de feocromocitomas y paragangliomas localizados, no invasivos e histológicamente benignos en nuestra institución entre los años 1960 y 1976.Ochenta y ocho pacientes tenían feocromocitoma/paraganglioma no familiar, de tipo esporádico. Nueve tenían el síndrome de neoplasia endocrina múltiple (NEM) tipo 2 (2A: 7, 2B: 2) y uno feocromocitoma familiar. Setenta y nueve pacientes tenían feocromocitoma único; 10 tenían tumores extra adrenales (paragangliomas) y 9 tumores adrenales multicéntricos o bilaterales.Seis pacientes (6.5%) desarrollaron feocromocitoma recurrente después de haberse documentado niveles postoperatorios normales de catecolaminas; uno de ellos tenía el síndrome NEM 2A. Las recurrencias se desarrollaron a intervalos de 5 a 13 años después de la resección inicial, distantes en 3 pacientes, locales en 2, y locales y distantes en sólo un paciente. Ninguna de las recurrencias ocurrió en los 13 pacientes que en la revisión histopatológica presentaban invasión local o vascular. Ningún paraganglioma exhibió recurrencia.El seguimiento de por vida en pacientes que hayan tenido feocromocitomas o paragangliomas es mandatorio.

Ectopic ACTH syndrome. Diagnostic and therapeutic aspects.

Twenty-five patients were identified with non-pituitary, nonadrenal ACTH-secreting tumors (bronchial carcinoid, bronchial small cell carcinoma, pancreatic islet cell carcinoma, medullary thyroid carcinoma, thymic carcinoids, metastatic adenocarcinoma, and pancreatic cystadenoma). Clinical features were weakness, hypertension, cushingoid appearance, peripheral edema, personality disorders, and hyperpigmentation. Biochemical features were a markedly increased urinary free cortisol level (all patients), hypokalemia (71 percent of patients), and an elevated ACTH level (72 percent of patients). Surgical therapy consisted of bilateral total adrenalectomy (56 percent of patients). Twelve percent underwent transsphenoidal hypophysectomy and 36 percent had excision of their tumor. No surgical therapy was undertaken in 28 percent. Bilateral total adrenalectomy in patients with a slow-growing malignancy or an unknown tumor secreting ACTH is beneficial in relieving symptoms and prolonging life. Excision of nonmalignant ACTH-producing tumors yields an excellent long-term prognosis.

Long-term consequences of intraoperative spillage of bile and gallstones during laparoscopic cholecystectomy

Laparoscopic cholecystectomy is associated with a higher incidence of iatrogenic perforation of the gallbladder than open cholecystectomy. The long-term consequences of spilled bile and gallstones are unknown. Data were collected prospectively from 1059 consecutive patients undergoing laparoscopic cholecystectomy over a 3-year period. Details of the operative procedures and postoperative course of patients in whom gallbladder perforation occurred were reviewed. Long-term follow-up (range 24 to 59 months) was available for 92% of patients. Intraoperative perforation of the gallbladder occurred in 306 patients (29%); it was more common in men and was associated with increasing age, body weight, and the presence of omental adhesions (each P < 0.001). There was no increased risk in patients with acute cholecystitis (P = 0.13). Postoperatively pyrexia was more common in patients with spillage of gallbladder contents (18% vs. 9%; P < 0.001). Of the patients with long-term follow-up, intra-abdominal abscess developed in 1 (0.6%) of 177 with spillage of only bile, and in 3 (2.9%) of 103 patients with spillage of both bile and gallstones, whereas no intra-abdominal abscesses occurred in the 697 patients in whom the gallbladder was removed intact (P < 0.001). Intraperitoneal spillage of gallbladder contents during laparoscopic cholecystectomy is associated with an increased risk of intra-abdominal abscess. Attempts should be made to irrigate the operative field to evacuate spilled bile and to retrieve all gallstones spilled during the operative procedure.

Carcinoma of the colon and rectum complicating chronic ulcerative colitis.

Patients with pancolonic chronic ulcerative colitis are at increased risk to develop carcinoma of the colon. Controversy continues, however, as to whether this carcinoma is more “virulent” than “type ordinaire” carcinoma of the colon and as to the best way to manage these patients. This study reviews the characteristics and survival of 70 patients with cancer of the colon superimposed on chronic ulcerative colitis. Patients with carcinoma identified incidentally during prophylactic colectomy for chronic ulcerative colitis fared well (5-year survival of 72 per cent), while those with clinical symptoms or radiographic suggestion of cancer had a poor survival rate (5-year survival of 35 per cent). Patients with panproctocolitis, 10 years of disease, and early onset of disease are most likely to have cancer superimposed on chronic ulcerative colitis. This cancer is likely to have a poorer prognosis than type ordinaire cancer of the colon. Prophylactic proctocolectomy should be considered before evidence suggesting carcinoma develops.

Gastric adenocarcinoma complicating Gardner's syndrome in a North American woman.

Gastric carcinoma has not been recognized as a complication of Gardners syndrome in the Western world. We report a 37-yr-old white woman with Gardners syndrome who had gastric adenocarcinoma in association with diffuse gastric polyps. At the time of laparotomy, the disease was metastatic to regional lymph nodes. In addition, the patient had mesenteric and retroperitoneal fibromatosis, thyroid adenomas, a chest wall hemangioma, and fibrocystic disease of the breast. This is the first reported case of gastric adenocarcinoma complicating Gardners syndrome in North America.

Endoscopic variceal sclerosis: a one-year experience

Our first years experience with endoscopic variceal sclerosis (EVS) performed with a cuffed balloon fiberoptic endoscope is presented. Seventy-five patients have been treated with EVS without requiring the use of general anesthesia. All but one patient had endoscopically documented variceal hemorrhage and underwent EVS while bleeding or shortly thereafter. Ninety-two per cent of the patients were discharged from the hospital after initial treatment with the current overall survival rate being 89.3%. Complications resulting in a prolonged hospitalization were infrequent, but two deaths occurred in which EVS complications may have been a contributing factor. Based on these results, EVS is recommended for managing variceal hemorrhage and is a reasonable alternative for shunt surgery in the management of variceal bleeding.

Adenoacanthoma of the colon

SummaryThe clinical and pathologic features of five cases of adenocanthoma of the colon are presented. Although the lesion is rare, both the pathologist and the surgeon should be aware of this unusual neoplasm in order to avoid error in diagnosis, especially when metastatic lesions are being evaluated. In our experience, these rare colonic lesions are more aggressive than ordinary carcinomas of the colon.

The pathology and surgical management of primary hyperparathyroidism.

Parathyroid pathology is an inexact science, and especially in parathyroid surgical procedures, the surgeon has to be the judge of what is significantly abnormal.

Does the avoidance of nasogastric decompression following elective abdominal colorectal surgery affect the incidence of incisional hernia

PURPOSE: In a previous, prospective, randomized study of the use of nasogastric tubes in patients undergoing elective abdominal colorectal surgery, we found that patients who did not have nasogastric (NG) decompression postoperatively had a significantly higher rate of abdominal distention, nausea, and vomiting. Patients from that study have now been followed for a median duration of 5.3 years to evaluate whether this elevation in perioperative intra-abdominal pressure would subsequently lead to an increased incidence of incisional hernia. RESULTS: Of the 251 patients who received NG decompression, 8 (3.2 percent) developed incisional hernias compared with 15 (6.6 percent) of 229 patients who were not decompressed (P=0.085). CONCLUSIONS: The increase in postoperative abdominal distention and vomiting that occurs in patients who do not receive NG decompression does not lead to a significantly increased incidence of incisional hernia. Furthermore, we continue to support avoidance of routine prophylactic post-operative nasogastric decompression in uncomplicated, elective abdominal colorectal surgery.

Pediatric endocrine surgery

The pediatric surgeon is in a unique position to understand endocrine surgery and, therefore, is expected to develop considerable expertise in this area. In recent years numerous advances and changes have occurred in pediatric endocrine surgery that have led to greater understanding of the disease processes and syndromes and the development of new diagnostic techniques and surgical approaches.