Is glucagonoma of the pancreas a curable disease?

BACKGROUND: Glucagonomas are rare neuroendocrine tumors of the pancreas. Because of its rarity, its natural history is not well understood. AIM: We evaluated the natural history of glucagonomas treated at a tertiary care cancer center. METHODS: A retrospective analysis of 12 patients during 1970 to 2000 was performed. Six patients (50%) had a tumor located in the head of the pancreas. RESULTS: Abdominal pain (83%) and weight loss (75%) were the most common symptoms. Median tumor size was 6 cm (range 0.04-10). Seven patients (58%) had liver metastases. Five patients (42%) underwent curative resection. Overall median survival was 66 mo, and 5-yr overall survival was 66%. Five-yr overall survival was 83% for patients who had resection versus 50% for the non-resected patients (p = 0.04). Patients who were disease-free had a complete resection of the primary tumor and no liver involvement. CONCLUSIONS: Glucagonomas generally present with liver metastases at the time of diagnosis. Cure is only possible if the disease is localized and completely resected.

Repeat hepatic resection for recurrent colorectal cancer.

Recurrence in the liver following hepatic resection for metastatic colorectal carcinoma is a predictable phenomenon, occurring in about two-thirds of patients who develop recurrence. There are few data, however, about the value of repeated hepatic resection in patients who have a recurrence in the liver following initial resection of their hepatic metastases. We have reviewed our experience with 10 patients (of whom 9 were evaluable), culled from a series of 74 patients who had an initial hepatic resection for metastatic colorectal carcinoma. There were seven men and two women, mean age 52 (range 34-75 years). Duke's stages of the primary cancer were B1 in two patients, B2 in one patient, and C2 in six patients. Most of the patients had elevated carcinoembryonic antigen (CEA) and constitutional symptoms as indications for the second-look procedure. There was one surgical death due to hepatic failure in a patient who required a trisegmentectomy. The average interval between the first and second hepatic resections was 21 months. The estimated 1- and 5-year actuarial survivals from the second liver resection were 78% and 23%, respectively. The median survival was 41 months from the first resection (range 14-100 months) and 16 months from the second resection (range 0-92 months). In conclusion, repeat hepatectomy for recurrent liver metastases is a viable option for the well selected patient. It is a low risk surgical procedure and may augment survival in the patient with well documented metastases limited to the liver.

Current perspectives on repeat hepatic resection for colorectal carcinoma: a review.

BACKGROUND: Recurrence occurs in 65% to 85% of patients after initial hepatectomy for metastases from colorectal cancer. Approximately one half of these have liver metastases, and in 20% to 30% only the liver is involved. Opportunity for resection is frequently limited because of diffuse liver disease or extrahepatic extension, and only 10% to 25% of these patients have conditions amenable to resection. This current review is focused on the rationale, indications, and results of resection of hepatic metastases from colorectal cancer. METHODS: The major series of liver resection were reviewed, and the cases of repeat resections were culled out. In addition to standard clinical parameters, the indications and timing after initial resection and the survival and subsequent recurrence after repeat resection were recorded. RESULTS: A comprehensive review of the 28 series showed that the mean interval between the first and second liver varied from 9 to 33 months and was about 17.5 months in the two largest series. The median survival in series reporting 10 or more patients was 19 months (mean, 24 months), which is comparable to data in single resection series. In the large French Association series containing 1626 patients with single resections and 144 patients with two resections, the 5-year survival was 25% and 16%, respectively. The recurrence rate after repeat resection is high (greater than 60%), and one half are in the liver. The prognostic factors favoring repeat resection are variable, but they include absence of extrahepatic extension of tumor and a complete resection of the liver metastases. CONCLUSIONS: Repeat hepatic liver resection for metastatic colorectal cancer in carefully selected patients appears warranted in view of reasonable survival expectations, which approach that of single liver resection. Risk of recurrence is high, however, suggesting the need for rigorous preoperative and intraoperative assessment and postoperative adjuvant therapy

Patient selection for hepatic resection of colorectal metastases.

OBJECTIVE: To determine the major factors governing patient outcome after hepatic resection of metastatic colorectal cancer and to formulate criteria for optimal resection. PATIENTS: We reviewed records of 74 patients (44 men, 30 women) who underwent resection of colorectal liver metastases. MAIN OUTCOME MEASURES: Sex, age, primary tumor location; Dukes tumor stage; disease-free interval after primary resection (synchronous vs metachronous); location, number, size, and distribution of liver metastases; operative complications; and mortality. RESULTS: The primary tumor location was rectosigmoid in 46 patients and the colon in the others. The tumor stage was Dukes A in one patient, Dukes B in 16, Dukes C in 31, and Dukes D (synchronous metastases) in 26. The disease-free interval was less than 12 months in 38 patients and 12 months or more in 36. The location of the metastases was the right lobe in 42 patients, left lobe in 22, and bilateral in seven. The cancer was unilobar in 55 patients and bilobar in 18. Surgical resection included wedge resection in 23 patients, segmentectomy in 30, lobectomy in seven, and trisegmentectomy in 12. The number of lesions resected was one in 50 patients, two or three in 18, and four or more in five. Nine patients had repeated liver resections because of recurrence. There were five postoperative deaths within 60 days (7%), four of which occurred after extended resection and were complicated by delayed liver failure and multisystem failure. An additional death occurred at 65 days after an apparently uneventful initial convalescence. Overall median survival was 35 months; actuarial 5- and 10-year survival rates were 24% and 12% respectively. There were significant relationships with survival (P<.05) for the number of metastases (three or fewer vs four or more), bilobar vs unilobar metastases, and extent of liver resection (wedge and segmental vs lobectomy and trisegmentectomy). A multiple logistic regression model (multivariate analysis) showed a significant correlation with survival (P<.05) for distribution of metastases (bilobar vs unilobar) and extent of resection (wedge and segmental vs lobectomy and trisegmentectomy). CONCLUSION: Patient selection for hepatic resection of colorectal cancer metastases based on standard clinical and tumor outcome variables should be expected to achieve long-term survival with low morbidity and mortality in bilobar disease or extended resection should generally be avoided, especially in medically compromised patients.

Thrombotic thrombocytopenic purpura and HIV infection.

We report on a man who was HIV-seropositive and who was initially admitted following multiple episodes of syncope. He gradually developed fulminant thrombotic thrombocytopenic purpura (TTP). Twenty-one patients with TTP who were HIV-positive have been reported previously. Of these 22 patients, all treated with plasmapheresis, 7 died from TTP and 3 suffered relapse but eventually recovered. Delay in initiating plasmapheresis may be fatal. We have correlated the laboratory data of these patients at time of admission with subsequent clinical outcome; only the platelet count correlated with outcome. Patients with lower platelet counts were less likely to relapse or die with therapy. Physicians caring for patients infected with HIV should always consider the possibility of TTP in those patients with thrombocytopenia of unknown etiology. Review of the peripheral blood smear, allowing the detection of microangiopathic hemolytic anemia, is an important clue, enabling one to consider the correct diagnosis.

Cisplatin as a radiation sensitizer in the treatment of advanced head and neck cancers. Results of a phase II study.

BACKGROUND: Surgery and radiotherapy mainstays in the management of advanced head and neck cancer, although historically, only 20-30% of patients survive. Therefore, in an attempt to improve locoregional control and survival, a multimodal protocol using cisplatin as a radiosensitizer was implemented. METHODS: Between 1984 and 1990, 68 patients with advanced head and neck cancer (Stages III and IV) were treated with a regimen consisting of an induction phase of 4500 cGy and two cycles of cisplatin followed by an eradicative phase of either radical surgery (Group A, 27 patients) or radical radiotherapy (Group B, 41 patients). The maintenance phase chemotherapy consisted of adjuvant 5-fluorouracil (5-FU) and cisplatin after completion of locoregional treatment. Of the 68 patients, 19 had Stage III disease, and 49 had Stage IV; 21 had no regional lymph node metastases (N0), and 47 had regional lymph node metastases (N+). RESULTS: The induction phase yielded a 26% (18 patients) complete response (CR) rate and a 57% (39 patients) partial response (PR) rate (response > 50%), yielding an overall response rate of 83%. Eleven patients (16%) had stable disease (ST) (i.e., < 50% response). The 2-year survival rates by initial treatment response for patients who had a CR, a PR, and stable disease were 53%, 56%, and 36%, respectively; for Groups A and B, 63% and 45%, respectively; for Stages III and IV, 68% and 43%, respectively; and for N0 and N+, 69% and 43%, respectively. In Group A, 14 of 27 patients (52%) had no viable tumor in the surgical specimen (i.e. had pathologic complete tumor clearance [CTC]); this subgroup had a 5-year survival rate of 58%. Ten patients (37%) who had gross total resection of tumor with negative margins but had tumor present in the specimen had a 5-year survival of 22%. In Group B, the 5-year survival rate was 43% for 27 patients who achieved CR after completion of radical radiotherapy (total tumor dose, 6480-7020 cGy). The 5-year survival rate of the 14 patients who had a PR and stable disease after radical radiotherapy and 3 patients whose resection was incomplete was 0%. The overall 2- and 5-year survival rates for all patients were 53% and 32%, respectively. Of 21 patients in whom treatment failed, most (90%) had a locoregional recurrence: 13 local recurrences (62%), 5 regional (24%), and 1 locoregional (5%). Two patients (10%) experienced failure at distant sites (the lung). Major treatment-related morbidity developed in two patients. CONCLUSIONS: Although induction chemotherapy-radiotherapy produces a high clinical response rate, this does not translate into improved survival compared with historical controls. A subgroup that showed complete tumor clearance (CTC or pathologic complete response) at surgery had an apparent improved survival and merits further study. Patient selection did not appear to be a factor for the CTC group, because the majority of patients in this group had partial responses to induction therapy, nodal disease and advanced tumor stage, and tumor presence in unfavorable sites.

Giant condyloma acuminatum (Buschke-Lowenstein tumor) of the anorectal and perianal regions. Analysis of 42 cases.

PURPOSE: Giant condyloma acuminatum or Buschke-Loewenstein tumor of the anorectal and perianal regions is an uncommon entity that has not been extensively reviewed. We analyzed 42 known cases of giant condyloma acuminatum in the English literature and reviewed their behavior and management. METHODS: All reported cases of giant condyloma acuminatum in the English literature were selected. The relevant clinicopathologic features of this uncommon entity were examined and discussed. RESULTS: These tumors are generally large with the propensity to ulcerate and infiltrate into deeper tissues. The hallmark of the disease is the high rate of recurrence (66 percent) and malignant transformation (56 percent). No distant metastases have been reported. The overall mortality was 20 percent, all occurring in patients with recurrences. Fifty percent of the patients who were initially treated with radical surgery developed recurrences. The average duration of disease was longer in patients with recurrences than in patients without recurrences (9.6 years vs. 2.8 years). The median number of recurrences was two (range, one to seven) recurrences, and the median time before first recurrence was ten months. Recurrences were treated by radical surgery in 17 patients and chemoradiotherapy +/- local excision in 5 patients. Follow-up information for the remaining five patients was not available. The cure rate in the radical surgery group was 61 percent compared with 25 percent in the chemoradiotherapy +/- local excision group. CONCLUSIONS: Giant condyloma acuminatum of the anorectal and perianal regions is a highly aggressive tumor with the propensity for recurrences and malignant transformation, but without metastatic potential. A high rate of recurrence is seen in patients with long duration of the disease. Salvage of patients with recurrences can be achieved successfully with radical surgery.