Possibilities and limits of surgical therapy of pseudomyxoma peritonei

In a retrospective study, the potential and limitations of surgical therapy of pseudomyxoma peritonei were studied in seven patients. In all patients the pseudomyxoma had originated from the appendix. All patients were primarily treated by surgery. An R0 resection at the first operation was possible in only one patient with a benign pseudomyxoma, while significant tumor debulking with improved symptoms was achieved in all other patients. If the tumor recurred relaparotomy was performed to obtain tumor reduction. The perioperative morbidity even after multiple relaparotomies was low. The survival rates ranged between 2 and 20 years with chemotherapy (5-fluorouracil) which was of particular prognostic benefit in patients with malignant pseudomyxoma peritonei. Surgical therapy is the treatment of choice in pseudomyxoma peritonei, although an R0 resection is hardly feasible. Due to the low morbidity, relaparotomy in cases of tumor recurrence always appears to be indicated. In comparison to other gastrointestinal malignancies, the survival rates in pseudomyxoma peritonei, sometimes treated with additive chemotherapy, are superior.     

Intraoperative EMG response of the musculature after stimulation of the glenohumeral joint capsule

The application of open intraoperative intraperitoneal chemotherapy following cytoreductive surgery for the treatment of pseudomyxoma peritonei or peritoneal carcinomatosis requires safety precautions for the medical and non-medical personnel. In agreement with already existing rules, precautions were established which result in an optimum of safety. These concern the preparation of the cytostatic drugs, the application in the operating room as well as personal precautions intra- and postoperatively. After the establishment of theses recommendations, 22 patients were treated with open intraperitoneal chemotherapy in 1.5 years without any severe accidents. Therefore, a safe intraoperative use of cytotoxic drugs is possible. At the moment, the indication for such an approach may be given in peritoneal carcinomatosis from appendix, colon or ovarian cancer. In the future, an adjuvant application in other gastrointestinal malignancies (e.g. T3/T4 gastric carcinoma) may be considered.     

Successful treatment of pseudomyxoma peritonei using combination chemotherapy of intraperitoneal low-dose CDDP and oral 5'-DFUR administration

We report a case of pseudomyxoma peritonei treated with combination chemotherapy. A 73-year-old woman was diagnosed as having psuedomyxoma peritonei originated from the vermiform appendix. Appendectomy was performed, and 10 mg of MMC was administered intraperitoneally. From day 7, 600 mg/day of 5'-DFUR was orally given for 2 years, and 20 mg of CDDP once a month was intraperitoneally administered seven times. The patient has been doing well with no evidence of tumor recurrence for two years after operation. Combination chemotherapy with CDDP and 5'-DFUR, as a biochemical modulation therapy, has fewer side effects and leads to better quality of life of patients. We recommend a combination chemotherapy with low-dose CDDP and 5'-DFUR for patients in poor general condition.     

Three cases of Pseudomyxoma peritonei treated with intraperitoneal inoculation of cisplatin through an implantable port system]

Three patients with Pseudomyxoma peritonei were treated with intraperitoneal chemotherapy through an implantable port system which had been used subcutaneously. Three patients were given 100 mg of cisplatin for intraperitoneal chemotherapy and adjuvant chemotherapy was needed for only one of them. An adjuvant chemotherapy through an implantable port is an easy method and a useful therapy.     

Unusual tumors of the appendix and pseudomyxoma peritonei

This report covers hyperplastic and neoplastic lesions of the human vermiform appendix that, although unusual, are likely to be encountered by general surgical pathologists. Topics include epithelial neoplasms, hyperplastic polyps, diffuse epithelial hyperplasia, pseudomyxoma peritonei, goblet cell and tubular carcinoid tumors, neuroma, and neurogenous hyperplasia. We discuss recent advances in the prognosis and management of appendiceal carcinoma, current controversies regarding the relationship between ovarian and appendiceal epithelial neoplasms, the concept of the mucinous tumor of uncertain malignant potential (UMP), and practical aspects of reporting pseudomyxoma peritonei. Classical carcinoid tumors are beyond the scope of this article, and primary neoplasms that are so rare they occur in the literature only as isolated case reports are not covered.     

Pediatric liver tumors

An infant or child who presents with a large intrahepatic mass will most likely have a malignant tumor. In children, benign tumors constitute only 30% of liver tumors and most are vascular in origin. Treatment of benign vascular tumors is conservative and seldom surgical. Hepatoblastoma is the most common malignant tumor followed by hepatocellular carcinoma. Treatment of malignant tumors is based on a combination of surgery and chemotherapy. Children with hepatic malignancies that can be resected have an excellent prognosis. Other rare benign and malignant tumors of the liver do occur and surgery plays a critical role in management.     

In vitro MR microscopy of the hippocampus in Alzheimer''s disease

In prepubertal children, teratoma is a benign tumor, whereas in adolescents and adults, it behaves as a malignant neoplasm. Adult patients without evidence of metastases may be candidates for surveillance after orchiectomy, and those with low-volume or borderline retroperitoneal metastases thought to contain teratoma might best be managed by surgery, as teratomatous deposits do not respond to chemotherapy. Patients with larger retroperitoneal metastases may be given chemotherapy before surgery to reduce or eliminate other germ-cell elements. Teratomatous masses persisting after chemotherapy are excised by most clinicians, in part to obtain a pathologic diagnosis. In childhood tumors, inguinal orchiectomy or enucleation is sufficient if one is certain the lesion contains only teratoma.     

Adjuvant radiotherapy and chemotherapy for stage II or IIIA non-small-cell lung cancer after complete resection. Provincial Lung Cancer Disease Site Group

GUIDELINE QUESTIONS: 1) Does the use of postoperative, adjuvant radiotherapy or chemotherapy, alone or in combination, improve survival rates among patients with completely resected, pathologically confirmed stage II or IIIA non-small-cell lung cancer (NSCLC)? 2) Does the use of radiotherapy reduce the risk of local recurrence among patients with completely resected stage II or IIIA NSCLC? OBJECTIVE: To make recommendations about the use of postoperative adjuvant radiotherapy and chemotherapy in the treatment of patients with completely resected stage II or IIIA NSCLC. OUTCOMES: Overall survival and disease-free survival are the primary outcomes of interest. A secondary outcome of interest is local disease control. PERSPECTIVES (VALUES): Evidence was collected and reviewed by 4 members of the Lung Cancer Disease Site Group (Lung Cancer DSG) of the Cancer Care Ontario Practice Guidelines Initiative. The evidence-based recommendation resulting from this review was approved by the Lung Cancer DSG, which comprises medical oncologists, radiation oncologists, pathologists, surgeons and a medical sociologist. A community representative was present at 1 meeting during which the recommendation was discussed. QUALITY OF EVIDENCE: One meta-analysis and 22 randomized controlled trials (RCTs) were published between 1962 and 1996. The RCTs compared surgery plus radiotherapy with surgery alone; surgery plus adjuvant chemotherapy with surgery alone; surgery plus radiotherapy with surgery plus both chemotherapy and radiotherapy. Many studies included patients with stage IIIB NSCLC; some included patients with incompletely resected stage I NSCLC or with small cell lung cancer (maximum 10%). Older studies used chemotherapy or radiation that would now be considered inferior according to current standards of practice. BENEFITS: There was no survival benefit with adjuvant radiotherapy alone, although 3 RCTs reported a reduction in the rate of local recurrence among patients treated with adjuvant radiotherapy. The meta-analysis showed that postoperative, cisplatin-based chemotherapy alone reduced the relative risk of death by 13% (hazard ratio [HR] 0.87, 95% confidence interval [CI] 0.74 to 1.02); in combination with radiotherapy it resulted in a 6% reduction in the relative risk of death (HR 0.94, 95% CI 0.79 to 1.11). HARMS: Postoperative adjuvant chemotherapy with alkylating agents was found in the meta-analysis to increase the relative risk of death by 15%. A study involving prolonged adjuvant chemotherapy (busulfan or cytoxan daily for 2 years) reported that 4 of 726 patients had hematologic malignancies. In 1 study, only 53% of patients received all 4 cycles of chemotherapy with cyclophosphamide-doxorubicin-cisplatin (CAP); in another, 22% of patients refused therapy with CAP because of nausea and vomiting. PRACTICE GUIDELINE: There is evidence from RCTs that postoperative radiotherapy reduces rates of local recurrence by 11% to 18% (or 1.6 to 19-fold) among patients with completely resected, pathologically confirmed stage II or IIIA NSCLC. Therefore, if the outcome of interest is a reduction in the frequency of local tumour recurrence, radiotherapy is recommended. However, there is no evidence of a survival benefit from postoperative radiotherapy alone. In a meta-analysis, postoperative chemotherapy with or without radiotherapy resulted in a slightly reduced (statistically nonsignificant) risk of death among patients with surgically resected stage II or IIIA NSCLC. The survival benefit was small and achieved only with chemotherapy regimens that produced substantial toxic effects and that are no longer used. Newer chemotherapy regimens are currently being evaluated as adjuvant therapy, but there is insufficient evidence of benefit at this time to recommend them. Therefore, if the outcome of interest is survival, there is insufficient evidence to recommend current chemotherapy regimens with or without radiotherapy as postoperative, adjuvant the     

The approach to the patient with single and multiple liver metastases, pulmonary metastases, and intra-abdominal metastases from colorectal carcinoma

Recurrent colorectal carcinoma constitutes a major health care problem, with 90,000 patients diagnosed annually with metastatic disease. Recent advances have offered treatment to selected patients with liver, lung, and intra-abdominal metastases. Resection of liver secondary tumors improves 5-year survival from 0% to approximately 30% and offers the only possibility for cure. As experience mounts, hepatic surgery can be performed with quite acceptable morbidity and mortality. Adjuvant therapies are being developed that may improve results with surgery alone. Cryoablation is a new technique that appears to effectively eradicate liver tumors, but its role remains to be defined. In patients with unresectable disease, the benefit of hepatic artery infusion of chemotherapy is unproven. Resection of pulmonary metastases significantly improves survival in patients with solitary nodules. Consistent data regarding the benefit of pulmonary metastatectomy in patients with multiple nodules are not available. Combined cytoreductive surgery and intraperitoneal hyperthermic chemotherapy is being investigated as a treatment for peritoneal carcinomatosis from colorectal cancer. Although selected patients may benefit, this combined treatment modality appears to be less effective in patients with colorectal cancer than with other types of cancer.     

Optimal treatment in gestational trophoblastic disease

Gestational trophoblastic diseases are a heterogenous group of conditions ranging from the benign hydatidiform mole to the malignant choriocarcinoma. Optimal therapy in this group of diseases rest in the correct diagnosis, assessing their risk for malignant behavior using prognostic scoring systems and administering appropriate treatment. Their rarity makes it imperative that these patients are treated in special centres by experts. Benign moles are treated surgically with evacuation of the uterus or hysterectomy. In malignant gestational trophoblastic disease, chemotherapy is the treatment of choice; single agent for non-metastatic and low-risk metastatic disease and combination chemotherapy for high-risk metastatic disease. Judicious use of surgery and radiotherapy in these cases will improve the survival rate. With appropriate treatment, the cure rates approach 100% in the low-risk group and 80% to 85% in the high risk group.     

Testicular morphological changes in children with acute lymphoblastic leukemia following chemotherapy

Morphological changes in the testis induced by chemotherapy given according to the Tokyo Children's Cancer Study Group (TCCSG) regimens were studied in children with acute lymphoblastic leukemia (ALL). After informed consent, testicular biopsies were performed 14 times in 12 patients at the end of treatment. The testicular morphology in all cases had sustained a degree of damage. The tubular fertility index (TFI), calculated as the percentage of seminiferous tubules containing identifiable spermatogonia, was from 0 to 42.8% (mean 33.4%) below the normal value. Infiltration of leukemic cells was the most significant factor contributing to the decrease in TFI. There were no differences in the TFI among the TCCSG protocols. Formation of sperm was recognized in six cases, whose ages were 7, 8, 9, 10, 15 and 19 years. In two children, testicular biopsy was performed twice. In the second biopsy, TFI was elevated and sperm formation with the maturation of Leydig cells was observed. A number of other pathological changes were observed: modification of spermatogonia, Sertoli cells and inclusion bodies in spermatogonia, abnormal maturation of Leydig cells, evidence of interstitial fibrosis and thickening of the basement membrane. These results suggest that recent strong chemotherapy for the treatment of ALL might cause severe but not fatal damage to children's testicular tissue. As chemotherapy escalates, more investigation of testicular function will be necessary.     

Combined chemotherapy and radiotherapy, followed or not by surgery, in squamous cell carcinoma of the esophagus

BACKGROUND: This study was designed to evaluate the feasibility of a neo-adjuvant combined chemo-radiotherapy in patients with localized squamous cell carcinoma of the esophagus. PATIENTS AND METHODS: Forty-two patients with squamous cell carcinoma of the esophagus, stages II and III (or stage I if considered to be poor candidates for immediate curative surgery), age less than 70 years and WHO performance status 0 to 2, were enrolled in a study of radiotherapy combined with chemotherapy, consisting of 2 (operated patients) or 3 (nonoperated patients) courses of cisplatin, vindesine, mitomycin-C or cisplatin, vinblastine. Surgery was routinely proposed to patients. RESULTS: Thirty-seven patients (88%) received full preoperative therapy. Of 30 patients responding to this preoperative therapy, 12 had a third cycle of treatment and 15 had esophagectomy. Three of the operated patients had no pathological evidence of residual tumour. Median survival of all 42 patients is 11 months and the 2-year survival rate is 29%. There is no difference in survival among responding operated or non-operated patients. Our group represents 95% of all eligible cases of squamous cell carcinoma of the esophagus occurring in Geneva during the study period. CONCLUSION: Our series gives a realistic view of the median survival of a population of patients eligible for neo-adjuvant therapy of esophagus cancer, and suggests that secondary surgery might not improve the patient survival. Furthermore, non-selected patients are at high risk for therapy-related death.     

Current status of surgery for mediastinal tumors]

Impact of surgery on the result of mediastinal tumors is variable: In benign tumors such as teratoma, congenital cyst, intrathoracic struma and neurogenic tumor, a complete resection causes a good result. Simultaneous thoracotomy and laminectomy should be performed in the Dumbbell type of neurogenic tumor. Since complete resection of the invasive thymoma promises a good prognosis, excision of all the tumor and invading tissues followed by radiation therapy is recommended in the treatment of thymoma. In pure seminoma resection and radiation therapy is the treatment of choice, while in non-seminomatous germ cell tumor, aggressive chemo- and radiation therapy should be performed, until tumor markers such as AFP and HCG become negative. The operation is to be followed afterwards. A mass screening test for neuroblastoma in infant facilitates an early detection of this disease and promises a good result by chemotherapy, radiation therapy, and surgery. There is still a possibility that the result of treatment of the malignant mediastinal tumors would be improved by exact diagnosis and intensive treatment.     

Kidney-specific expression of a novel mouse organic cation transporter-like protein

OBJECTIVE: To assess the anterior mediastinal mass in recurrent testicular cancer, with relation to thymic hyperplasia after treatment. METHODS: The anterior mediastinal regions were fully evaluated by chest computed tomography (CT) at the initial staging and after treatment in 24 of 44 patients with testicular cancer. RESULTS: One patient with stage IIB tumor had thymic hyperplasia before treatment, and one with stage III had benign thymic hyperplasia after chemotherapy with salvage surgery. Three of 4 patients who had recurrence had an anterior mediastinal mass. One had benign thymic hyperplasia confirmed by histology and 2 had metastatic tumor confirmed by histology and clinical course, in which the mass became so enlarged that it obstructed major vessels. CONCLUSION: Although the relationship of the CT finding to the response to treatment in the anterior mediastinal mass and other metastatic lesions provide some clues helpful in differentiating benign from malignant masses, surgical exploration is recommended for the patient with an indication for salvage surgery.     

Thyroxine suppressive therapy in patients with nodular thyroid disease

PURPOSE: To review evidence about thyroxine suppressive therapy in patients with thyroid nodules, including the clinical importance and natural history of nodules and the effects and potential side effects of thyroxine therapy. DATA SOURCES: English-language articles published from 1986 to December 1996 were identified through searches of the MEDLINE database, selected bibliographies, and personal files. DATA EXTRACTION: Randomized, controlled trials and nonrandomized trials of thyroxine suppressive therapy for solitary and predominantly solid thyroid nodules were reviewed. In most studies, nodule cytology was evaluated by fine-needle aspiration biopsy. Therapy was considered suppressive if suppression was documented by thyroid-stimulating hormone-releasing hormone tests or sensitive thyroid-stimulating hormone assays. Response was defined as a decrease of 50% or more in nodule size or volume; most recent studies measured nodule size by ultrasonography. DATA SYNTHESIS: The evidence suggests that thyroxine suppressive therapy fails to shrink most nodules: Only 10% to 20% of nodules responded to this treatment. Fine-needle aspiration biopsy is more reliable in distinguishing benign from malignant nodules. Recent studies suggest that spontaneous decrease in size with complete disappearance of thyroid nodules is not uncommon. No data show that thyroxine therapy arrests further growth in most existing nodules or prevents the emergence of new nodules. Postoperative thyroxine therapy does not seem to prevent recurrence of thyroid nodules except in patients with a history of radiation therapy. Potential adverse effects of long-term suppressive therapy include osteoporosis and heart disease. CONCLUSIONS: Patients with cytologically benign nodules are best followed without thyroxine treatment. Most benign nodules remain stable in size and remain benign when monitored for a long time. For nodules that increase in size, biopsy should be done again or surgery should be performed.     

Audit of colorectal cancer surgery by non-specialist surgeons

BACKGROUND: Some authorities recommend that colorectal cancer should be treated in specialist units but evidence that non-specialist units demonstrate comparatively poor results may be lacking. METHODS: Between 1987 and 1991, 267 patients were operated on by four general surgeons, none of whom was a specialist in colorectal surgery. Procedure-related complications, postoperative mortality and disease-related survival rates were analysed. RESULTS: There were four cases of intraperitoneal sepsis (1 per cent) and five of 189 patients (3 per cent) had clinical anastomotic dehiscence; there was no case of wound dehiscence. The postoperative mortality rate after elective and emergency surgery was 2 and 13 per cent respectively. The 5-year disease-related survival rate for curative and palliative surgery was 67 and 9 per cent respectively. There were no significant differences between the surgeons. CONCLUSION: Disease-related variables such as early-stage disease and fewer patients presenting as emergencies may have a greater favourable influence on ultimate survival than surgeon-related variables.     

Primary plasmacytoma of the lymph node (plasmocytic lymphoma). The differential diagnosis of nodal plasma-cell proliferates

HISTORY AND CLINICAL FINDINGS: A 60-year-old man was found on routine examination to have an enlarged, firm, cervical lymph node. He looked older than his age and his general condition was poor. He had no fever, nocturnal sweating or weight loss. Further examination revealed no hepatosplenomegaly on palpation, but numerous enlarged cervical lymph nodes were palpable. INVESTIGATIONS: Histological investigation of a cervical lymph node revealed a marked increase in slightly pleomorphic plasma cells with monotypic expression of IgM-kappa. Multiple myeloma was excluded on the basis of histological and cytological findings in the bone marrow. Serology revealed a mild antibody deficiency syndrome (gamma-globulin 7.8%) with signs of acute inflammation and an increase in alpha 2-globulin. There was no evidence of a monoclonal gammopathy on electrophoresis. A diagnosis of primary nodal plasmacytoma was made. COURSE: As the patient was asymptomatic at diagnosis he was not given chemotherapy. There was no evidence of tumour progression at follow-up examination two months later. His progress will be monitored closely. CONCLUSION: This patient's history is consistent with the prognosis generally associated with primary nodal plasmacytoma that is much better than that of multiple myeloma.     

Strategies to decrease the incidence of intra-abdominal recurrence in resectable gastric cancer

Two main approaches are suggested to improve treatment results in resectable gastric cancer: extended lymphadenectomy and adjuvant antitumour therapy. Progress is to some extent stalled by the perception of gastric cancer as a pathophysiologically uniform disease; it has been demonstrated, however, that there are variants of gastric cancer associated with predominantly intra-abdominal spread or with haematogenous metastases. Recent clinicopathological studies have provided information about the mechanisms of this metastatic diversity. A review of clinical trials suggests that no single method of treatment can efficiently address all variants of gastric cancer spread, but new treatment strategies may be based on defining the pathophysiological variant of gastric cancer and selecting adjuvant therapy according to the most probable mode of tumour spread. Treatment should start with surgery which includes a 'reasonably' extended lymphadenectomy aimed at achieving an increased rate of curative resection and more accurate staging. Risk factors for peritoneal spread of tumour require the perioperative use of intraperitoneal chemotherapy. Subsequent adjuvant therapy may be indicated in patients at high risk of further cancer spread or occult metastases, as determined by pathological examination of the resected specimen.     

Low back pain in children and adolescents: to treat or not?

Recent cohort data has shown that low back pain is a common symptom in adolescents that, by the age of 16, approaches the level found in adults. The symptoms are frequently recurrent, but are not usually associated with disability. Spells are frequently forgotten, and medical attention is not generally sought. Although the possibility of serious spinal pathology must be considered, the majority of adolescent back trouble may be considered a normal life experience. The efficacy of treatment for non-specific back pain in this age group is undetermined, but the similarities with adult symptoms suggests that management should follow current clinical guidelines for adults (early activation and advice stressing the benign nature of the problem). Persisting root pain may best respond to chemonucleolysis. There is no evidence that treatment or lifestyle changes at this age will reduce symptoms in adult life, but inappropriate medical attention may have detrimental psychosocial consequences.     

Dissent over standards in adjuvant management of colon carcinoma

Since 5-fluorouracil (5-FU) plus levamisole substantially reduces the recurrence rate and improves survival in adjuvantly treated patients with curative resected stage III colon cancer this combination has been considered the standard therapy. Shortly thereafter folinic acid modulated 5-FU-therapy also demonstrated adjuvant efficacy compared to surgery alone. Therefore various schedules of folinic acid modulated 5-FU-therapy were compared with the standard regimen (5-FU/levamisole). Randomized multicentric studies revealed: In three of four studies 5-FU/FA is superior to 5-FU/levamisole. Treatment duration of 6 months for 5-FU/FA is similar to 12 months 5-FU/Levamisole. No benefit is obtained for 5-FU/FA by additional levamisole. The effect of regional (portal vein or intraperitoneal) treatment is controversial discussed, but combination of the treatment with systemic chemotherapy versus 5-FU/levamisole demonstrated slightly increased therapeutic efficacy. Immunotherapy with autologous tumor cell-BCG or monoclonal antibody treatment improved survival and is currently investigated in studies with conventional systemic treatment and combined chemoimmunotherapy. Beside treatment in studies patients with colon cancer stage III should be offered adjuvant chemotherapy with 5-FU/FA. Further improvements and adjuvant treatment protocols for stage II carcinoma have to be investigated in studies.