Heerden, Jonathan A. van

Abstract Occasionally pancreatoduodenectomy is performed for clinically suspected pancreatic malignancy only for the surgeon to find that a benign aetiology accounts for the pancreatic mass. The aim of this study was twofold: to determine the incidence of pancreatoduodenectomy performed for a misdiagnosis of pancreatoduodenal malignancy and to identify potentially avoidable errors in preoperative and intraoperative judgement. Between 1956 and 1990, radical pancreatoduodenectomy was performed in 603 patients at the Mayo Clinic; 29 (5 per cent) underwent pancreatoduodenectomy for a diagnosis made before and during surgery of primary pancreatic or periampullary malignancy that was later proven histopathologically to be either unsuspected subacute or chronic pancreatitis (22 patients), benign fibrous common bile duct stricture (two), and penetrating duodenal ulcer, metastatic melanoma, ampullary adenoma, intrahepatic drug-induced cholestasis and pseudocyst (one each). No patient had a preoperative diagnosis of chronic pancreatitis. Weight loss, pain and/or jaundice were present in 27 of the 29 patients. Objective imaging tests, such as computed tomography, ultrasonography and/or endoscopic retrograde cholangio-pancreatography, were performed in 25 patients. Potential errors in judgement may be avoided by a more aggressive attempt at biopsy in selected patients; in others, resection for presumed malignancy may be unavoidable.

AbstractIdentification of multiple clinical and pathologic prognostic factors in differentiated thyroid cancer has permitted some degree of risk stratification. However, these clinical indices fail to distinguish potential intrinsic differences in tumor virulence. The nm23 gene has been identified as a potential metastasis suppressor gene that is homologous to nucleoside diphosphate kinases. Studies in human breast cancer have shown a significant inverse correlation between nm23 levels and nodal involvement/tumor recurrence. Given the possible clinical utility of a marker of metastatic potential in the management of thyroid carcinoma, we examined 34 thyroid neoplasms and a human medullary thyroid cancer (MTC) cell line (TT) for nm23 expression. Normalized nm23 expression was assessed by Northern analysis of tumor RNA. nm23 Expression (tumor expression/TT cell expression, mean ±SE) was 1.14 ±0.15* in MTCs (n =5), 0.70 ±0.10* in follicular cancers (n=6), 0.51 ±0.11 in papillary cancers (n=19), and 0.31 ±0.03 in follicular adenomas (n=4) (*p< 0.05 when compared to adenomas). Within histologicgroups, we found no correlation between nm23 expression and nodal involvement or distant métastases. Our results indicate that thyroid neoplasms of different histologies express varying levels of the nm23 transcript. Although nm23 expression seems diminished in metastatic breast cancer, it appears not to be the case in metastatic thyroid cancer. The nm23 gene may therefore have different roles in the evolution and metastases of different neoplasms.

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.Eighty‐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.Six 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.Life‐long follow‐up of all patients who have had pheochromocytomas or paragangliomas resected is mandatory.

AbstractSymptomatic hypercalcemia is a medical emergency. Knowledge of the alteration in the factors that control normal calcium homeostasis permits selection of a plan of treatment, which includes general supportive measures along with therapeutic agents directed at the specific alteration in calcium homeostasis. The rationale for the selection of various modes of treatment and treatment schedules and the untoward side effects of the therapeutic agents are reviewed.Acutely developing hypocalcemia often requires emergency treatment. Relief of symptoms can be achieved with intravenous calcium preparations. Knowledge of the underlying cause permits selection of appropriate medications when long‐term treatment is required. When vitamin D is needed, measurement of serum calcium and phosphate at regular intervals is essential. Hypoparathyroidism may be temporary after thyroid or parathyroid surgery, and efforts should be made 6 to 12 months after operation to determine whether the abnormality is permanent.

Abstract Background Calciphylaxis is a rare, painful, life-threatening problem of cutaneous necrosis and refractory healing seen in patients with uraemia and secondary hyperparathyroidism. The pathogenesis involves abnormalities in calcium and phosphorus metabolism, and acute deposition of calcium in tissues. Methods The clinical course of 16 patients diagnosed with calciphylaxis at this institution from 1994 to 1998 was reviewed. Results Fourteen women and two men, with a mean age of 56 (range 39–70) years, presented with chronic renal disease of various causes, hyperparathyroidism and characteristic skin lesions. All patients underwent intensive medical therapy, including haemodialysis (n = 16), parathyroidectomy (PTX) (n = 7) and skin debridement of cutaneous lesions (n = 8). Mean preoperative serum values in surgical (PTX) versus non-surgical patients were: calcium 9·9 and 9·3 mg dl−1 (P = 0·25); phosphorus 5·8 and 4·9 mg dl−1 (P = 0·04); calcium–phosphorus product 61·6 and 45·2 (P = 0·03); and parathyroid hormone (PTH) 56·8 and 5·9 pmol l−1 (P = 0·0001) respectively. Mean postoperative values for calcium (8·7 mg dl−1), phosphorus (4·2 mg dl−1), calcium–phosphorus product (30·5) and PTH (4·0 pmol l−1) in surgical patients changed significantly (P &lt; 0·05). Median overall survival was 9·4 months with 15 patients now deceased. Median survival was 14·8 and 6·3 months for PTX versus no PTX (P = 0·22), 14·1 and 6·1 months for skin debridement versus no debridement (P = 0·08), 12·4 and 6·6 months for proximal versus distal skin lesions (P = 0·60), and 6·5 and 13·9 months for diabetic versus non-diabetic patients was (P = 0·11). Conclusion Calciphylaxis appears to have a female preponderance with a very dismal prognosis. A multidisciplinary approach using frequent haemodialysis to normalize serum calcium and phosphorus levels, and local debridement of skin lesions seems prudent. PTX cannot be recommended routinely in all such patients unless severe secondary hyperparathyroidism with poorly controlled metabolic disturbance mandates surgery. The exact indications for PTX for calciphylaxis remain unclear.