CASES--clinical audit; scenarios for evaluation and study

The incidence of bilateral involvement it is generally estimated to be 5% to 10%. It shows association with certain congenital anomalies and it has an increased occurrence of familial cases. The records of 9 children (5 boys, 4 girls) diagnosed at Vall d'Hebron Hospital with bilateral Wilms tumor between 1976-1995 were analyzed. Six patients had synchronous tumors and 3 had metachronous lesions. Genitourinary malformations were present in 4 children and another had hemihypertrophy. Two children were brothers. Eight patients underwent pre-operative radiation therapy and/or chemotherapy. Five patients had nephrectomy on one side (3 of them had metachronous presentation) and partial nephrectomy on the other side. The other children had bilateral partial nephrectomy or tumorectomy. Seven out of the nine patients are alive (78%). The two children who died presented with stage IV tumors and high grade malignant. One boy suffers cardiomyopathy. All survivors have normal renal function. With the proven efficacy of chemotherapy, bilateral renal salvage procedures were demonstrated to be effective in controlling disease without compromising renal function or survival. The innovative approaches developed for the treatment of bilateral Wilms tumor may influence the treatment of unilateral Wilms.     

Endometrial stromal sarcoma of the vulva arising in extraovarian endometriosis: a case report and literature review

OBJECTIVES: To better characterize intrarenal neuroblastoma. METHODS: We retrospectively reviewed the records of 5 children with neuroblastoma initially diagnosed as Wilms' tumor. RESULTS: Five patients, aged 11 months to 3.5 years, were preoperatively diagnosed as having intrarenal Wilms' tumor, prior to operative or histopathologic diagnosis of neuroblastoma. In 3 children the diagnosis of neuroblastoma involving the kidney was confirmed; in the fourth child subcutaneous metastatic disease precluded biopsy confirmation of the intrarenal tumor; and in the fifth child the kidney was severely displaced but not actually involved by neuroblastoma. All patients were hypertensive and 4 patients had elevated urinary catechol levels. Molecular genetic studies showed N-myc amplification in both patients who were evaluated. All patients had poorly differentiated histopathology. One patient is presently alive at 8 months with recurrent disease and another patient is free of disease 13 years after diagnosis. The remaining 3 patients died of the malignancy. CONCLUSIONS: Intrarenal neuroblastoma is a rare entity that clinically and radiographically may resemble Wilms' tumor. Our limited experience indicates that intrarenal neuroblastoma is an aggressive malignancy, and long-term survival is rare.     

Phototherapy for patients with Alzheimer disease with disturbed sleep patterns: results of a community-based pilot study

Twenty of 67 children registered on the International Registry of Childhood Adrenocortical Tumors between May 1988 and December 1994 had small adrenocortical tumors (defined for this study as measuring < or = 200 cm3 and/or weighing < or = 100 g). We reviewed the records of these 20 patients to characterize the clinical and pathologic findings and outcomes of children with small adrenocortical tumors. Median patient age was 2 years (range, 4 months to 5 years). There was only one boy. All had clinical signs of virilization, and seven had signs or symptoms of Cushing syndrome. A median 5.5 months (range, 1-40 months) had elapsed between the first signs of endocrine dysfunction and diagnosis. All tumors were surgically resected. Tumor volume was 3.3-195 cm3 (median, -8.7 cm3), and weight was 3.7-100 g (median, 36 gm Tumor samples were histologically reviewed in 18 cases. Eight were adenomas, and 10 were carcinomas (6 low grade and 4 high grade). Pathology records described tumor with diagnostic features of adrenocortical carcinoma in two patients. One patient received mitotane for 8 months after surgery. Only one patient had recurrent disease, which was detected 6 months after diagnosis and proved rapidly fatal. Another has been lost to follow-up. The remaining 18 patients are alive with no evidence of disease at a median 2.3 years (range, 6 months to 6.1 years) after diagnosis. Our data suggest that children with small adrenocortical tumors have an excellent prognosis with surgery as the sole therapy, regardless of tumor histiotype.     

Treatment of intracranial ependymomas of children: review of a 15-year experience

PURPOSE: There are still major controversies in the optimal management of children with intracranial ependymomas. To assess the impact of tumor site, histology, and treatment, the outcome of children treated at the Institut Gustave Roussy was reviewed retrospectively. METHODS AND MATERIALS: Between 1975 and 1989, 80 children aged 4 months to 15.8 years were seen at the Institut Gustave Roussy for postoperative management of an intracranial ependymoma. Location of tumor was infratentorial in 63 cases and supratentorial in 17. Surgical treatment consisted of complete resection in 38, incomplete resection in 38 and biopsy only in 4. Postoperative irradiation was done in 65 patients and chemotherapy in 33. Surviving patients have been followed from 12-197 months with a median of 54 months. RESULTS: The 5-year actuarial survival and event-free survival are 56% and 38%, respectively. Thirty-four patients relapsed from 3-72 months after diagnosis (median 25 months). In 20 patients, the only site of failure was the original tumor site. Three patients failed locally and at distance, while 10 others failed only at distance. Survival at 5 years was significantly better for patients who had complete resection of the tumor (75% vs. 41%, p = 0.001) and for those who received radiation therapy (63% vs. 23%, p = 0.003). Event-free survival at 5 years was superior in patients with complete resection of the tumor (51% vs. 26%, p = 0.002) and in patients who received radiation therapy (45% vs. 0%, p < 0.001). Sex and tumor site had no impact on survival or event-free survival. There was no difference in survival, event-free survival, or pattern of failure between patients treated with local field, whole brain or craniospinal irradiation, while severe longterm sequelae were noted predominantly in the latter two groups. CONCLUSION: Considering that failures were predominantly local and that there was no apparent benefit from prophylactic irradiation, we recommend local field irradiation with doses above 50.0 Gy for all children with intracranial ependymomas, without meningeal dissemination at diagnosis. Special considerations are necessary for children < 3 years of age.     

Malignant vascular tumors in young patients

BACKGROUND: To the authors' knowledge there are few published series of malignant vascular tumors in patients age < or = 21 years. METHODS: The authors retrospectively documented the clinical presentation, pathology, treatment, and outcome of patients age < or = 21 years with malignant vascular tumors treated between 1970-1995 at Memorial Sloan-Kettering Cancer Center. The histologic sections were rereviewed to confirm the diagnosis. RESULTS: Four patients were identified with angiosarcoma and two with malignant hemangioendothelioma. Five patients were female and one was male; the median age at diagnosis was 11.8 years (range, 8 months-21 years). The tumor involved the skin in one patient, soft tissue in one patient, bone in two patients, and internal organs in two patients. One patient had associated diffuse angiomatosis and another had the Klippel-Trenaunay-Weber syndrome. This patient received prior radiation therapy to the primary site with the subsequent development of a vascular sarcoma. None of the patients had distant metastases at diagnosis. Resection was attempted in five patients and completed in four. Chemotherapy alone was given to three patients whereas radiation therapy alone and radiation plus chemotherapy were administered to one patient each. The median follow-up was 4.9 years (range, 1 month-12 years). There were three deaths from progressive disease and two deaths from locoregional recurrences. Reexcision and radiotherapy controlled one local recurrence. Another patient developed recurrence to regional lymph nodes and further dissemination. The primary tumor in all three survivors was excised completely whereas two of the three patients who died of progressive disease underwent an incomplete excision or biopsy alone. CONCLUSIONS: Malignant vascular tumors are rare in the first two decades of life and when they do occur are very aggressive. Complete resection is curative for patients with localized lesions.     

Esthesioneuroblastoma. Long-term outcome and patterns of failure--the University of Virginia experience

BACKGROUND: Esthesioneuroblastoma is a rare tumor arising from olfactory epithelium. This retrospective review analyzed the patterns of failure and long term outcome of patients with esthesioneuroblastoma evaluated at a single institution. METHODS: Forty patients with esthesioneuroblastoma were evaluated at the University of Virginia, with a median follow-up of 130 months. In most cases, treatment consisted of combined-modality therapy, including radiotherapy and surgery for Stages A and B disease and the addition of chemotherapy for Stage C disease. Fifteen patients received chemotherapy that included cyclophosphamide plus vincristine. Thirty-eight patients received radiotherapy, with a median dose of 50 Gy. Initial surgery for 23 patients included craniofacial resection, whereas the remainder had less extensive surgery (3 had no initial surgery). Five patients were salvaged with high dose chemotherapy and autologous bone marrow transplantation (CTX/BMT). RESULTS: Actuarial survivals at 5, 10, and 15 years are 78%, 71%, and 65% respectively. Fifty-five percent of patients failed therapy, and 68% of the failures were locoregional. Thirty-nine percent of recurrences occurred later than 5 years from diagnosis. Three of the five patients were successfully salvaged with CTX/BMT compared with four of seventeen patients who underwent conventional salvage therapy. CONCLUSIONS: Esthesioneuroblastoma is associated with long term survival and late recurrences. Multimodality therapy should be used initially. Durable remissions of failures can be achieved with CTX/BMT:     

Epithelioid smooth-muscle tumors of the uterus: a clinicopathologic study of 18 patients

Epithelioid smooth-muscle tumors of the uterus are uncommon neoplasms for which prognostic factors have not been well established. A retrospective follow-up study of 18 uterine epithelioid smooth-muscle tumors was performed. Patients ranged in age from 27 to 83 years (mean, 45 years) and were separated into three groups based on the nuclear grade of the epithelioid tumor cells. Two tumors had grade 1 nuclei; both were examples of intravenous leiomyomatosis. They had highest mitosis counts of 1 and 3 mitotic figures (MF)/10 high-power fields (HPF), no tumor cell necrosis was found, and both patients were alive with no evidence of disease at 64 and 5 months' follow-up. Ten tumors had grade 2 nuclei. All had highest mitosis counts of 0 to 3 MF/10 HPF, except one (5 MF/10 HPF). Tumor cell necrosis was absent in nine and only one had an infiltrative border. Tumor size ranged from 1.5 to 14 cm. Two tumors contained pleomorphic ("symplastic") multinucleated giant cells similar to those seen in bizarre leiomyomas. All nine patients with follow-up were alive with no evidence of disease 5 to 203 months postoperatively (median, 74 months). One patient had also received adjuvant radiation therapy. Six tumors had grade 3 nuclei. Highest mitosis counts of 4 to 9 MF/10 HPF were found in five; one had 1 MF/10 HPF. Maximum tumor size ranged from 4.5 to 13 cm. Two had tumor cell necrosis, and two had an infiltrative border. Two of these patients died of tumor 11 and 132 months postoperatively. The former had widespread metastases at initial surgery (stage IVb); the latter patient (stage I) developed the first of seven tumor recurrences 3 years postoperatively. Both patients had also received adjuvant chemotherapy. Of the remaining four patients, two were alive with no evidence of disease at 48 and 83 months, one was alive (tumor status unknown) at 28 months, and one was lost to follow-up. Based on our findings and those in the literature, we conclude that uterine smooth-muscle tumors with a predominance of epithelioid cells are extremely uncommon and metastasize infrequently. No single histologic feature is predictive of metastatic potential. Clinically malignant tumors (i.e., epithelioid leiomyosarcomas) typically have the combination of significant nuclear atypia (either grade 2 or grade 3 nuclei) and some mitotic activity (usually at least 3 to 4 MF/10 HPF); most also have tumor cell necrosis.     

Development of transgenic yellow poplar for mercury phytoremediation

We report a case of a teratoid Wilms tumor arising within a supernumerary ectopic ureteropelvic structure in a 7-year-old boy. The tumor was near the right kidney but was completely separate from it. On the contralateral side, the child had a duplication of the ureteral system. Pathologic examination revealed the mass to be completely enveloped by a fibromuscular sac lined by urothelium. The tumor had a botryoid, polypoid architecture and, in addition to areas of classic Wilms tumor, had extensive squamous, mucinous, and columnar elements. Teratoid Wilms tumors are rare and, to the best of our knowledge, have not previously been described as arising either in anomalous urinary structures or at 7 years of age.     

Localized malignant mesothelioma. A clinicopathologic and flow cytometric study

Six examples of malignant mesothelioma appearing as a localized pleural mass are described. There were four women and two men, ranging in age from 42 to 76 years. A history of asbestos exposure was obtained from three patients. The tumors ranged in size from 2.8 to 10 cm. Two were pedunculated and four were sessile with broad-based pleural attachments. Histologically, three tumors were purely epithelioid and three were biphasic. Immunohistochemical stains in all six cases were positive for cytokeratin and negative for carcinoembryonic antigen. Five were also positive for epithelial membrane antigen. Five were negative for Leu-M1, while one showed focal staining in a peripheral membrane pattern. Electron microscopy in two purely epithelioid tumors showed long, thin microvilli, well-developed desmosomes, and numerous tonofilaments. Flow cytometry showed an aneuploid DNA content in four tumors and a diploid content in one. Flow cytometry in five cases identified a DNA aneuploid cell population in four tumors and a diploid population in one. Three patients showed signs of local recurrences 4, 7, and 18 months after excision and died of their disease 12, 10, and 24 months after diagnosis, respectively. Three patients are well with no evidence of disease 8, 24, and 96 months after diagnosis. These findings indicate that malignant mesotheliomas of the pleura may rarely appear as a localized mass. The biologic behavior of such tumors is difficult to predict, but some patients survive disease-free for a long time after surgical excision.     

Significance of routine annual esophagram for early detection of carcinoma of the esophagus

BACKGROUND/AIMS: In order to achieve increased survival rates for patients with carcinoma of the esophagus, early detection of the disease is vital. Serial esophagrams were evaluated to clarify which interval would be effective for early detection of carcinoma of the esophagus during routine examination. MATERIALS AND METHODS: One hundred eighty-nine patients with carcinoma of the esophagus were grouped into three, according to the experience and the time of the previous roentgenograms before the definite diagnosis. RESULTS: Five patients were in Group 1, in which roentgenographic examination had been done within 12 months prior to the diagnosis. Retrospective observation revealed a slight but certain abnormal shadow at the same location as the esophageal tumor seen on the second films. In Group 2, seven had received an esophagram between 12 and 24 months before the diagnosis. In contrast to Group 1, neither abnormality nor findings indicating esophageal tumors were detected on the former x-ray films, in all seven cases. Group 2 was characterized by relatively small tumors and low stage of the disease. Mean tumor length was 4.1 +/- 2.9 cm, and three of seven were classified as Stage I and two as Stage IIA. On the other hand, most of the 177 patients in Group 3, with no previous examination of the esophagus within 24 months before the diagnosis, had far advanced disease. Mean tumor length was 6.3 +/- 2.6 cm. Only nine (5.1%) were classified as Stage I, whereas 115 (65.0%) were classified as Stage III or IV. CONCLUSION: In light of these data, for populations in which esophageal cancer frequently occurs, esophageal examination every 12 months will no doubt contribute towards the early detection of lesions.     

Asynchronous bilateral retinoblastoma: the St. Jude Children's Research Hospital experience

Between May 1962 and July 1993, 172 children presented at the St. Jude Children's Research Hospital for evaluation and/or treatment of retinoblastoma (RB). Of these, 65 presented with bilateral disease, while 107 had unilateral tumors. Of these 107 patients, nine subsequently developed RB in the unaffected eye. Initial age at diagnosis of these nine patients ranged from 3 weeks to 24 months (median = 2 months); five of the nine had a family history of RB at the time of initial diagnosis and one patient, without a family history of RB, presented with unilateral multiple tumors indicating inheritance of a germinal mutation and increased risk of RB development in the companion eye. Time to development of companion eye RB was 1-61 months postinitial diagnosis. Treatment of the initial eye included enucleation (n = 4), chemotherapy (n = 3), irradiation (n = 7), or a combination of these three modalities. Reese-Ellsworth grouping of the companion eye disease included I A (n = 7), III A (n = 1), and IV A (n = 1). Treatment of the second affected eye included irradiation in seven patients, cryotherapy in four, and chemotherapy in three. No companion eye has required enucleation to date. At last followup, 14/18 eyes remain intact. There have been no cases of metastatic dissemination; however, one patient has developed a second malignant neoplasm outside the field of irradiation. Eight of the nine patients remain alive. This experience reinforces the need for close follow-up of patients diagnosed with unilateral RB, especially those with a family history of RB and those with unilateral multiple tumors.     

Ovarian neoplasms in children

OBJECTIVE: To review the clinical presentation, treatment, and outcome in a series of children with ovarian neoplasms. DESIGN: A retrospective review of the medical records in a case series of 29 girls with ovarian neoplasms. The length of follow-up ranged from 6 months to 7 1/2 years and averaged 3.0 years in the girls with malignant tumors. SETTING: The patients were treated at a large referral children's hospital. PATIENTS: Twenty-nine girls with ovarian neoplasms were treated from 1976 to 1992. The average age of the patients was 10 years and ranged from 2 to 16 years. MAIN OUTCOME MEASURES: The principal outcomes examined were mortality and surgical morbidity. RESULTS: The most common presenting symptoms for these ovarian tumors in pediatric patients included chronic abdominal pain, an abdominal mass, or distention. Three girls presented with precocious puberty or hirsutism. In 27 cases, the tumor was a primary ovarian lesion. In two patients, the ovarian mass was the presenting finding for a stage IV non-Hodgkin's lymphoma. Seventeen tumors were benign and 12 were malignant. Tumors originating from the germ-cell line predominated (n = 17). Seven of the 10 ovarian malignant neoplasms were stage I at the time of diagnosis. All but one of the girls with malignant tumors received either adjunctive radiation therapy or multiple-agent chemotherapy. Two girls with sex cord/stromal cell tumors who presented with stage I disease ultimately developed widespread metastases. Both girls with large epithelial tumors survived. All of the girls with benign tumors and seven (70%) of 10 with malignant lesions survived. CONCLUSION: Ovarian tumors are unusual lesions in the pediatric population. Unlike in adults, such neoplasms generally originate from the germ-cell line. Whereas most ovarian tumors in girls are benign, some children have malignant tumors that are very aggressive and do not respond well to adjuvant therapy. In particular, malignant sex cord/stromal cell tumors, even when they present at an early stage, may behave unpredictably.     

Cryptosporidium parvum--propagation of oocyst in neonatal calves

A retrospective analysis of treatment of 8 children aged 5 to 13 years with intracranial germ cell tumors (3 germinomas, 5 nongerminomatous cell tumors) is reported. All patients were initially operated. Five of them underwent total or incomplete removal of the tumor. After surgery 2 children received radiotherapy alone (1 germinome, 1 nongerminomatous germ cell tumors) and 5 (2 germinomas, 3 nongerminomatous germ cell tumors) combined radio- and chemotherapy. One patient (choriocarcinoma) didn't receive any adjuvant treatment after total tumor removal. All 3 children with germinomas show complete remission for 29, 40 and 93 months. Of 5 patients with nongerminomatous germ cell tumors in 2 the duration of remission was 22 and 53 months and 3 relapsed 4, 12 and 24 months after treatment. The data presented demonstrate that the treatment results of intracranial germ cell tumors in children depend on histologic type of the tumor. Thus, precise tumor type determination is most important for choosing optimally adequate treatment approach.     

Cantrell''s pentalogy: a case report

Nine chemotherapy-naive women with recurrent (2 patients) or high risk factors (bilateral or extraovarian spread, poorly-differentiated tumor, age > or = 40 years at diagnosis, residual disease after surgery) granulosa-cell tumors were treated with cisplatin, cyclophosphamide with or without doxorubicin (PAC, PC) or cisplatin, etoposide and bleamycin (PVP-16B). Toxicity was acceptable and the most frequently encountered adverse reactions were WHO grade 3 gastrointestinal toxicity in 77% of patients, and grade 3 myelosuppression in 22% of cases. Clinical complete response was achieved in the 2 patients with recurrent disease. Five patients underwent second look surgery which documented: complete response in 3 patients, partial response in 1 patient and progressive disease in 1 case. Median survival was 85 months (range 14-103). Cisplatin-based cytotoxic regimens may be of benefit in the treatment of recurrent or high risk granulosa-cell tumors.     

Cancer in human immunodeficiency virus-infected children: a case series from the Children's Cancer Group and the National Cancer Institute

PURPOSE: To describe the spectrum of malignancies in human immunodeficiency virus (HIV)-infected children and the clinical outcome of patients with these tumors. METHODS: We retrospectively surveyed the Children's Cancer Group (CCG) and the National Cancer Institute (NCI) for cases of cancer that occurred between July 1982 and February 1997 in children who were HIV seropositive before or at the time of cancer diagnosis. We used Kaplan-Meier survivorship curves, hazard function estimates, and Cox proportional hazards models to evaluate survival. RESULTS: Sixty-four children (39 boys, 25 girls) with 65 tumors were reported. Thirty-seven children (58%) acquired HIV infection vertically (median age at cancer diagnosis, 4.3 years); 22 children (34%) acquired HIV through transfusion of blood or blood products (median age at cancer diagnosis, 13.4 years). Forty-two children (65%) had non-Hodgkin's lymphoma (NHL). Eleven children (17%) had leiomyosarcomas (or leiomyomas), which are otherwise exceptionally rare in children. Other malignancies included acute leukemia (five children), Kaposi's sarcoma (KS; three children), Hodgkin's disease (two children), vaginal carcinoma in situ (one child), and tracheal neuroendocrine carcinoma (one child). Median survival after NHL diagnosis was 6 months (range, 1 day to 89 months) and after leiomyosarcoma was 12 months (range, 10 days to 19 months). The average monthly death rate after NHL diagnosis was 12% in the first 6 months, which decreased to about 2% thereafter. In contrast, the monthly death rate after leiomyosarcoma diagnosis increased from 5% in the first 6 months to about 20% thereafter. CONCLUSION: After NHL, leiomyosarcoma is the second leading cancer in children with HIV infection. Both cancers have high mortality rates; improved outcome for NHL, in particular, may depend on earlier diagnosis and therapy.     

Uveal metastasis from carcinoid tumor. Clinical observations in nine cases

BACKGROUND: Carcinoid tumor is a low-grade malignancy that usually arises in the gastrointestinal tract or bronchus and rarely metastasizes to the eye. Metastasis of carcinoid tumor to the uvea can be confused clinically with other primary and metastatic uveal tumors. METHODS: The authors reviewed the records of 410 consecutive patients with uveal metastases referred to the Ocular Oncology Service at Wills Eye Hospital to identify those in whom carcinoid tumor was the primary neoplasm. The authors evaluated the clinical features of these metastases. RESULTS: Of 410 consecutive patients with uveal metastases, the primary neoplasm was a carcinoid tumor in 9 (2.2%). There were four men and five women. The mean age at ocular diagnosis was 50 years. In five patients (56%), the primary tumor was undiagnosed at ocular presentation. In the other four patients, the mean time interval from diagnosis of the primary carcinoid tumor to uveal metastasis was 89 months (range, 55-180 months). The site of the primary carcinoid tumor was the bronchus in seven patients, the esophagus in one, and the thymus in one. The site of intraocular metastasis was the choroid in six patients, the ciliary body in two, and the iris in one. All choroidal tumors had a characteristic orange color. Initial ocular treatment included external beam radiotherapy in five patients, plaque radiotherapy in two, argon laser photocoagulation in one, and local resection in one. Ocular tumor control was achieved in each patient. After a mean follow-up of 34 months, four patients (44%) are still alive. Five patients have died, with a mean survival of 34 months (range, 2-104 months) after the diagnosis of uveal metastasis. CONCLUSIONS: Uveal metastasis from carcinoid tumor is rare and tends to arise from the bronchus. Clinically, it has a distinctive orange color and may be associated with a longer systemic survival, compared with uveal metastasis from other primary sites.     

Using thymus anatomy to dissect T cell repertoire selection

OBJECTIVES: To re-examine clear cell cystic lesions of the kidney and to assess their potential clinicopathologic significance, as the long-forgotten diagnosis of benign adenomatous multicystic kidney tumor (Perlmann's tumor) has not been cited in the literature in more than 35 years. METHODS: We identified 12 patients between 1959 and 1996 who underwent a radical nephrectomy at our institution and were diagnosed with either adenomatous multicystic clear cell kidney tumor (n = 4) or with renal cell carcinoma (RCC) associated with features of adenomatous clear cell multicystic kidney tumors (n = 8). All diagnoses were reviewed histologically by a single pathologist. RESULTS: Nine of 1 2 patients had Stage T2NOMO disease, and 3 patients had Stage T1 NOMO disease. There were 8 men and 4 women. The average age at the time of surgery was 60.5 years (range 25 to 74). Six patients are still alive with a mean follow-up of 4.7 years (range 1.5 to 16.3) and have no evidence of recurrent disease. Of the 6 patients who died, mean survival time was 8.8 years (range 0 to 15.7). One patient died in the perioperative period, and the other 5 patients died of other causes, unrelated to their kidney tumor. CONCLUSIONS: Adenomatous clear cell multicystic kidney disease may represent a histologically distinct benign neoplasm, and its presence in association with RCC may confer a more favorable prognosis. Its distinction from usual solid hemorrhagic or focally necrotic RCC is important.     

The use of nuclear morphometry to predict prognosis in pediatric urologic malignancies: a review

Wilms tumor, the most common pediatric urologic malignancy, and genitourinary rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, represent two of the most commonly diagnosed pediatric urologic malignancies. The introduction and use of multimodal therapy (surgery, radiation, and chemotherapy) by the National Wilms Tumor Study (NWTS) and the Intergroup Rhabdomyosarcoma Study (IRS) groups have greatly improved the survival among children with these malignancies. Present survival rates for Wilms tumor exceed 85% and for rhabdomyosarcoma survival rates are approaching 80% as well. For Wilms tumor, current treatment trends suggest less intense therapy for those children with favorable histology tumors who are considered at relatively low risk for tumor recurrence. Likewise, the significant morbidity associated with the present therapy regimens for rhabdomyosarcomas has prompted investigators to search for individualized management schemes for children with a high probability of responding. The need for accurate criteria to separate these high and low risk groups becomes imperative. In this review we present our work using nuclear morphometry, as a prognostic indicator, to retrospectively predict response to therapy for children with Wilms tumors and genitourinary rhabdomyosarcomas.     

Primary transitional cell carcinoma in vesical diverticula

OBJECTIVES: To evaluate the treatment and prognosis of primary tumors in bladder diverticula. METHODS: The cases of 611 patients treated for bladder tumors at a single medical center were retrospectively reviewed. RESULTS: Eight patients had primary intradiverticular transitional cell carcinoma. Five patients had Stage Ta tumor, and 3 had Stage T1 tumor. Most patients were treated by local resection and adjuvant intravesical chemotherapy. All patients with initial Ta disease are disease free at the time of this writing. One patient with T1 disease died, 1 patient's disease recurred several times, and 1 patient showed positive cytology without apparent disease. CONCLUSIONS: Superficial intradiverticular tumors may be treated conservatively. Routine cystoscopy for patients with a bladder diverticulum is warranted for early diagnosis of possible intradiverticular tumor.