Smooth muscle tumors of the appendix and colon: a collective review of the world literature.

In this collective review, we compiled all reported cases of smooth muscle tumors of the colon/appendix in the world literature from 1959 to 1989. Our goal was to make more accurate conclusions about these tumors based on our increased data pool.

Smooth muscle tumors of the rectum and anus: a collective review of the world literature.

In this collective review, we have compiled all the reported cases of smooth muscle tumors of the rectum/anus in the world literature from 1959 to 1989. Our goal was to increase the data pool of smooth muscle tumors by adding these new data to that previously collected from 1881 to 1959. We increased the pool for leiomyomas from 89 to 148 and that for leiomyosarcomas from 54 to 215. By doing this, we hoped to make more accurate conclusions about smooth muscle tumors based on this increased data pool. Some interesting findings included three cases in small children that were found in our recent review: a 2-year-old with a leiomyoma and two small infants, aged 12 days and 36 days, with leiomyosarcomas. Again, the findings were probably consistent with an increased data pool. We were also able to find several more cases involving the anal region. We found the highest incidence of leiomyomas to have increased by a decade from the 40-49 year age group to the 50-59 year age group, while among leiomyosarcomas, there was about equal incidence among the 50-59 and 60-69 age groups. We doubt that these represent actual changes in the demographics, but rather that these latter findings are more accurate based on the greater quantity of cases available to us. As a further example, we found no appreciable sex difference; however, we did find more cases reported in females. From our increased data pool, we were able to find 16 more cases that were described as dumbbell-shaped, compared to one that was reported before 1959. Palpable mass, hemorrhage, and pain/discomfort continued as the most common symptoms reported at presentation. With regard to size, the majority of leiomyomas were found to be less than 5 cm in diameter, closely followed by those 5-9 cm. The majority of leiomyosarcomas were 5-9 centimeters at discovery. Most cases of leiomyoma were treated by excision, while most cases of leiomyosarcoma were treated by abdominoperineal resection, a finding consistent with old data. We hope that this paper thoroughly reviews pertinent information about leiomyomas and leiomyosarcomas of the rectum/anus and, in doing so, serves to refresh a few memories, stimulate others, and teach a few.

Is prophylactic cranial irradiation indicated in small-cell lung cancer?

Although prophylactic cranial irradiation (PCI) is frequently used in the treatment of patients with limited-extent small-cell lung cancer (SCLC), its role remains controversial. One hundred fourteen SCLC patients with limited disease treated at Indiana University were retrospectively reviewed. Fifty-eight of 114 (51%) patients achieved a complete remission (CR) and were analyzed. Thirty-eight of these 58 CR patients received PCI (+PCI) and 20 of 58 CR patients did not receive PCI (-PCI). Twenty-six of 38 patients who received PCI subsequently relapsed. No patient initially relapsed in the CNS, although one patient had a brain metastasis following recurrence in the chest. Eleven of 38 patients who were treated with PCI survived for longer than 30 months and were considered long-term survivors. Seven of these 11 patients (63%) developed clinically significant neurological toxicity. Sixteen of 20 patients who did not receive PCI relapsed, but there was only one initial relapse in the CNS. Three additional patients who relapsed in the chest subsequently developed CNS metastasis. All responded to palliative radiation with improvement in their symptoms. The high incidence of CNS toxicity in the long-term survivors and the relatively infrequent incidence of isolated CNS recurrent in patients not subjected to PCI raise serious questions concerning the role, if any, of PCI in limited SCLC.

Etoposide and split-dose cisplatin in bronchogenic carcinoma.

The Hoosier Oncology Group (HOG) treated 13 patients with bronchogenic carcinoma with an innovative schedule of cisplatin and VP-16. Unexpected toxicity was noted, with five deaths secondary to granulocytopenia and septic shock and three episodes of renal failure. Despite early closure of this study, we conclude that this schedule of cisplatin and VP-16 results in greater toxicity than comparable dosages in a more routine schedule.

KRAS2 oncogene overexpression in myelodysplastic syndrome with translocation 5;12.

The factors that initiate and maintain the abnormal hematopoietic clone in the myelo-dysplastic syndromes (MDS) remain largely unknown. We describe a patient with MDS associated with an abnormal karyotype, 46,XY,t(5;12)(q31;p12). According to the FAB cooperative group classification, the patient was classified as chronic myelomonocytic leukemia. Because of the particular chromosomal translocation, the structure-function relationship of three genes relevant to the translocation breakpoints, CSF2, FMS, and KRAS2, was studied in bone marrow and peripheral blood lymphocytes in this patient. No major structural alterations were observed at these three genetic loci. Although the levels of expression of the CSF2 and FMS genes remained unaltered, the KRAS2 oncogene was overexpressed approximately six-fold in bone marrow cells from the MDS patient compared with normal donors. We postulate that the RAS oncogene activation may be instrumental in the genesis of MDS.

Management of the myelodysplastic syndromes.

It is important to make the correct diagnosis of MDS and to exclude very carefully all other disorders that may induce dysplastic features in the bone marrow. In patients without excess of bone marrow blasts, cytogenetics and in vitro bone marrow cultures may aid in making the correct diagnosis. MDS patients without excess of bone marrow blasts or symptomatic cytopenia or cytogenetic abnormalities associated with poor prognosis should be followed on a regular basis with sequential examinations of blood counts and bone marrow specimens. In the absence of obvious disease progression, ie, increasing cytopenia or increasing percentage of marrow blasts, patients should only receive supportive care. An increase in RBC requirements alone is insufficient reason to start cytotoxic therapy. Once progression of the disease has been well documented, cytotoxic treatment is indicated. There is no reason to delay treatment until these patients have progressed to overt AML. In patients over the age of 50, the best available therapy is low-dose cytarabine with a 30% probability of a good response; this therapy requires careful supervision and the availability of intensive supportive care. In patients under 50 years with progressive disease, or with clear evidence of a poor prognosis, allogeneic BMT is the therapy of choice if a HLA-identical sibling can be identified. In those patients who lack a HLA-identical sibling, intensive combination therapy is the treatment of choice and should preferably include high-dose cytarabine. Intensive consolidation therapy will be necessary for a durable remission. Trials with inducers of differentiation remain experimental. Results to date have been disappointing.

A variant t(X;15)(p11;q22) translocation in acute promyelocytic leukemia.

Nonrandom reciprocal translocations involving chromosomes #15 and #17 are characteristic anomalies in a great majority of cases of acute promyelocytic leukemia (APL). Other complex translocations in APL that invariably involve chromosome #17 also have been described. We describe a patient with clinical and morphologic characteristics of APL but with a previously undescribed acquired karyotype, t(X;15)(p11;q22). This is the first translocation in APL described in which chromosome #17 is not involved. Although a comparative structure/function analysis of potentially relevant genes to the translocation breakpoints in both t(X;15) and t(15;17) APL showed no major alterations, the enhanced expression of the c-Ki-ras oncogene observed in t(X;15) APL supports the concept of heterogeneity in APL at the cytogenetic and molecular levels.