Papillary squamotransitional cell carcinoma of the cervix: a report of 32 cases

Papillary carcinomas of the uterine cervix with transitional or squamous differentiation are rare tumors that often resemble transitional cell carcinomas of the urinary tract. We reviewed 32 such cases of papillary cervical carcinoma and divided them into three groups: 1) predominantly (> 90%) squamous (nine cases), 2) mixed squamous and transitional (16 cases), and 3) predominantly transitional (seven cases). Overall, the patients ranged in age from 22 to 93 years (mean 50), and the most common clinical presentation was abnormal bleeding (15 patients) and an abnormal Papanicolaou smear (nine patients). The tumors ranged in size from 0.7 to 6.0 cm (mean 3.0). All cases demonstrated a papillary architecture with fibrovascular cores lined by a multilayered, atypical epithelium resembling a high-grade squamous intraepithelial lesion of the cervix. Underlying superficial to deep stromal invasion was seen in 18 of 20 cases (90%); in the remaining 12 cases, the specimen was too superficial to assess invasion. Eighteen (86%) of the 21 cases examined immunohistochemically demonstrated immunoreactivity for cytokeratin 7, whereas only two of the 21 (9.5%) showed positivity for cytokeratin 20. Of the 12 women for whom follow-up information was available, three were treated by simple hysterectomy, two underwent radical hysterectomy, one was treated with radiation alone, and one with combination chemotherapy and radiotherapy. Three patients died of disease (two in the squamous group and one transitional) within an average of 13 months after diagnosis. Local recurrence developed in two women, and one of these, a vaginal recurrence, occurred 12 years after the original diagnosis. Based on the above findings, we believe that these tumors are a clinicopathologically distinct, homogeneous group that display a morphologic spectrum. Nevertheless, because some tumors may show a purely squamous or purely transitional appearance, we propose retaining the above three separate designations for these tumors with the understanding that there is often a substantial degree of subjectivity in deciding whether a tumor is squamous or transitional. The most distinctive, objective, and easily recognizable feature of these tumors is their surface papillary architecture rather than their superficial resemblance to transitional cell carcinomas of the urinary tract, and we emphasize the need to distinguish these potentially aggressive malignant tumors from the far more common and benign papillary lesions of the cervix.     

High-Na+ low-K+ UW cold storage solution reduces reperfusion injuries of the rat liver graft

Pure sarcomas of the uterine cervix are rare; most of those reported have been leiomyosarcomas or rhabdomyosarcomas. Minimal data exists on malignant nerve sheath tumors in this site; only one typical example and one melanocytic example have been reported. We report three additional examples here in three patients 25, 65, and 73 years of age. The two older patients had vaginal bleeding and underwent hysterectomy as initial treatment. The youngest patient initially underwent only polypectomy. The tumors were 1.3, 4.4, and 5.0 cm in greatest dimension. The tumors were red-grey to white: two were polypoid and the third was ulcerated. The dominant microscopic appearance was that of cellular fascicles of spindle cells with hyperchromatic nuclei and eosinophilic cytoplasm. However, hypocellular areas were striking in each case; the hypocellular areas were fibromatous in two tumors and two had areas with a myxoid stroma (prominent in one). One tumor focally had cellular aggregates with a swirling pattern within a hypocellular background. Epithelioid foci in which tumor cells were rounded with conspicuous eosinophilic cytoplasm were focally prominent in one case. Mitoses were readily identified in each case. All three tumors were positive for S-100 protein and vimentin and negative for cytokeratin. HMB-45, and desmin. One case is recent and one patient had multiple metastases in the abdomen 2 years after hysterectomy. The patient treated initially by polypectomy underwent repeat local excision, followed by cone biopsy with positive margins, and then hysterectomy. She is clinically free of disease 15 months after diagnosis. Although the diagnosis of malignant schwannoma was suggested by the histologic features of the tumors, other diagnoses were entertained and immunohistochemistry was crucial in confirming the diagnosis. These tumors should be distinguished from other malignant spindle cell tumors of the cervix, such as leiomyosarcoma, endocervical "stromal sarcoma," and spindle cell melanoma, so their features, behavior, and optimal therapy can be further delineated.     

Brain tumors in children under 2 years: treatment, survival and long-term prognosis

Survival for children with brain tumors less than 2 years of age at diagnosis is dismal, and the quality of life of long-term survivors poor. Between 1975 and 1987, 78 (13%) of 579 patients with brain tumors treated at Children's Hospital of Philadelphia were under 2 years of age. Tumor site was posterior fossa in 31 (40%) and supratentorial in 47 (60%). Nine of 37 patients (24%) with malignant tumors, and 30 of 41 (73%) patients with benign tumors are alive with a mean follow-up of 116 months. Long-term survival after treatment with chemotherapy alone occurred in 10 patients, including 3 with malignant tumors. In 5 additional patients, chemotherapy delayed the need for irradiation a mean of 30 months. Of the 29 patients who relapsed after initial therapy, 12 are alive without progressive disease (2 patients with malignant tumors and 10 with benign tumors) a mean of 80 months after relapse, 2 children are alive with progressive disease, and 14 died a median of 48 months (range 9-115 months) after relapse. Twenty-one of the 39 survivors have minimal or no neurological or intellectual dysfunction. Surviving patients treated with surgery and chemotherapy have better intellectual function than patients treated with surgery and radiation (with or without chemotherapy) in that 8 of 10 children treated with surgery and chemotherapy have normal or above normal intelligence compared with 5 of 12 children receiving irradiation before their second birthday.     

Surgical treatment of primary neurogenic mediastinal tumors

In the years 1968-1989 out of 300 patients with surgically treated mediastinal tumors in 63 (21%) they were of neurogenic origin. Eighteen of these (29%) proved to be malignant. These tumors occurred in 23 males and 40 females, age range 14 months-67 years (mean 36 years). In all 45 patients with benign tumors and in 12 (66.7%) with malignant tumors the tumors were totally resected. In 4 patients the tumor was only partially resected in further two only a biopsy of the lesion was taken. During postoperative period one patient with a malignant tumor died. Ten (71.4%) patients survived 5 years with malignant lesions, while only 8 (61.5%) survived ten years. All patients that underwent resection of the benign tumor survived 10 years.     

'Progressive' versus 'indolent' idiopathic membranous glomerulonephritis

There is still controversy about safe and effective therapy for idiopathic membranous glomerulonephritis (MGN). Over 20 years, we have simply observed our patients clinically after diagnosis, and only used aggressive therapy with steroids in high dosage and azathioprine for 21 patients with progressive renal failure. The other 42 were thus classified as 'indolent' MGN. Those with 'progressive' MGN had heavier proteinuria and worse renal function on presentation, but the overlap was considerable. Patients with progressive MGN were treated after 1-4 years. All responded promptly, and 5 years after presentation all were alive, and only one was on dialysis. By 10 years, most were still alive, and of these most were off dialysis. In five patients, dialysis was delayed by several years. There were two deaths on dialysis, and three other deaths, mostly in older patients. All but one patient with indolent MGN remained stable on symptomatic treatment only, for at least 5 years after presentation. In many, proteinuria fell to insignificant levels over 4 years. In these remitting patients, there was a prevalence of thyroid disease (7), rheumatoid disease (3) and nephrotic presentation in pregnancy (4). After 6-10 years three patients developed worsening proteinuria and renal failure. Five older patients died from unrelated causes.     

Malignant tumors of the kidney, brain, and soft tissues in children and young adults with the tuberous sclerosis complex

BACKGROUND: The tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by seizures, mental retardation, and benign tumors of the brain, heart, skin, and kidney. Malignant tumors also can occur in patients with tuberous sclerosis, particularly in the kidney, although they occur less frequently than benign tumors. The types of malignancy that occur in TSC have not been characterized fully. METHODS: Clinical and pathologic features of 8 malignant tumors from 6 TSC patients ranging in age from 22 months to 21 years are reviewed. Six tumors were renal, one was from the inguinal region, and one was from the brain. The tumors were analyzed for loss of heterozygosity (LOH) in the chromosomal regions of the TSC1, TSC2, and VHL genes. RESULTS: Three patients (ages 7, 8, and 20 years) had renal cell carcinomas (RCCs). Two of these patients had multifocal RCCs. All three patients with RCC also had prominent multifocal dysplasia of renal cyst epithelium. Two patients (ages 20 and 21 years) had malignant angiomyolipomas (1 renal and 1 inguinal). One patient (age 22 months) had a Grade 4 giant cell astrocytoma (glioblastoma multiforme). LOH in the region of the TSC2 gene was found, either in the malignant tumor or in benign tumors, in all five patients whose DNA could be analyzed. CONCLUSIONS: Children with TSC, as well as adults with the disease, are at risk for developing malignant tumors. Two types of renal malignancy occur in TSC: RCC, which appears to arise from dysplastic renal cyst epithelial cells, and malignant angiomyolipoma. Tumors cytologically similar to malignant angiomyolipomas also may occur at extrarenal sites. LOH analyses suggest that the majority of patients with TSC who develop malignant tumors have germline TSC2, rather than TSC1, gene mutations.     

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.     

Human enteric pathogens identified in a London teaching hospital and a rural public health laboratory: 1994

BACKGROUND: Fetal rhabdomyomatous nephroblastoma (FRN) is a rare variant of Wilms tumor. MATERIALS AND RESULTS: One hundred and thirty two children with kidney tumors were seen at our hospital from 1985 to 1993. Among them were 6 (4.5%) who had FRNs. Five were boys aged 8 months to 3 years; the girl was 17 months old. Three of the four with unilateral disease had tumors so large that they were considered unresectable at diagnosis. Five received pre-operative chemotherapy and three also received pre-operative radiation therapy. None of the tumors responded. Both patients with bilateral tumors died of progressive disease. Three of the four patients with unilateral disease followed for at least one year are alive for 1 to 10 years after diagnosis. CONCLUSIONS: FRN should be in the differential diagnosis of huge kidney tumors in children, and preoperative therapies escalated with caution since FRN is not responsive to treatment used for classic Wilms tumor.     

Analysis of the predictive value of clinical data in patients with suspected colonic disease

OBJECTIVE: To develop guidelines for predicting colonic disease on the basis of clinical parameters. EXPERIMENTAL DESIGN: A prospective study of the clinical data prior to colonoscopy. On the basis of the endoscopic findings, the patients were divided into three diagnostic groups: absence of significant disease, significant benign disease and malignant disease. The patient population was divided randomly into two subgroups. The clinical data from one of them was used to build a database which, using Bayes' theorem, was compared with the variables from the other subgroup to predict the diagnosis for each patient. PATIENTS: A total of 336 patients (170 males and 166 females; mean age: 58 years; range: 15 to 87 years) were evaluated. RESULTS: When the endoscopic findings were grouped on the basis of their clinical importance, 211 patients (63%) belonged to the group without significant disease, 60 patients (18%) had significant benign disease and 65 (19%) presented a neoplastic disease. Of the 21 variables selected for use in the database, 6 showed statistically significant differences in terms of the absence or presence of malignant disease: age, absence of previous similar episodes, weight loss, rectal bleeding, lack of improvement and the presence of a mass on digital rectal examination. The predictive model differentiated patients with neoplasm from those without malignant disease, but was not capable of identifying differences among the latter. The model was useful for assessing the risk of malignant disease for each patient. CONCLUSIONS: The predictive model obtained is a useful tool for establishing the diagnosis and the priority in the performance of colonoscopy.     

Blood vessel transfer allowing avoidance of surgical rotation or amputation in the management of primary malignant tumors of the knee

PURPOSE OF STUDY: The surgical and clinical outcome of a limb-salvaging technique for tumors about the knee utilizing autogenous vessel transfer is reported. The original indication for these lesions was amputation or Van Nes rotationplasty because of vascular encasement by the tumor. MATERIAL: Twelve patients are reported: seven females and five males with an average age of 16 years. There were nine distal femur and three proximal fibula stage IIB primary malignant tumors. METHOD: All patients underwent a local wide resection of the primary tumor including the main vessels, followed by local reconstruction of the bone and vessels by an ipsilateral vessel transfer. Postoperatively the limbs were immobilized in a cast and the patients were treated with intravenous Heparin, followed by a single low dose Heparin injection for three months. RESULTS: Immediate postoperative arterial occlusion in one patient was successfully treated, but eight weeks later another occlusion occurred and an amputation was necessary. In another patient, arterial thrombi necrotized two toes; they were subsequently amputated. Other local complications were hematoma (two patients), superficial skin necrosis (one patient), and deep infection (one patient). All were successfully revised surgically. All resections obtained negative margins, and there were no local recurrences or distant metastases. In all twelve patients the tumor either encased the artery and vein, or the pseudocapsule of the tumor touched the vascular adventitia. DISCUSSION: The results show that by patients in whom there is attachment of the tumor with major vessels or in whom the vessels are encased by the tumor, wide limb-saving resection can be achieved by en-bloc resection of the primary tumor and the vessels. In this respect a contamination of the vessels is no longer a contra-indication for a limb-saving procedure. CONCLUSION: The described method is safe, has a relatively low complication rate comparable with rotationplasties and allows a limb-saving procedure.     

Resection, biopsy, and survival in malignant glial neoplasms. A retrospective study of clinical parameters, therapy, and outcome

Between July, 1984, and October, 1988, 263 patients (163 male, 100 female), aged from 4 to 83 years (mean 52 years), with malignant brain gliomas underwent surgical procedures: stereotactic biopsy in 160 and resection in 103 patients. There were 170 grade IV astrocytomas, 17 grade IV mixed oligoastrocytomas, 44 grade III astrocytomas, 22 grade III mixed oligoastrocytomas, and 10 malignant oligodendrogliomas. Overall median survival time was 30.1 weeks for grade IV gliomas, 87.7 weeks for grade III gliomas, and 171.3 weeks for malignant oligodendrogliomas. Multivariate analysis in 218 newly diagnosed cases revealed that the variables most strongly correlated with survival time were: tumor grade, patient age, seizures as a first symptom, a Karnofsky Performance Scale score of less than 70%, tumor resection, and a radiation therapy dose greater than 50 Gy. The proportions of patients receiving tumor resection versus biopsy in each of these prognosis factor groups were similar. Since most of the 22 patients with midline and brain-stem tumors were treated with biopsy alone, these were excluded. Considering 196 newly diagnosed patients with cortical and subcortical tumors, grade IV glioma patients undergoing resection of the contrast-enhancing mass (as evidenced on computerized tomography and magnetic resonance imaging) and postoperative external beam radiation therapy lived longer than those undergoing biopsy only and radiation therapy (median survival time 50.6 weeks and 33.0 weeks, respectively; Smirnov test, p = 0.0380). However, survival in patients with resected grade III gliomas was no better than in those with biopsied grade III lesions (p = 0.746). The authors conclude that, in selected grade IV gliomas, resection of the contrast-enhancing mass followed by radiation therapy is associated with longer survival times than radiation therapy after biopsy alone.     

Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases

The authors analyzed 153 cases of histologically verified intracranial germ cell tumors. The histological diagnosis was germinoma in 63 patients (41.2%), teratoma in 30 (19.6%), and other types of tumors in 60 patients (39.2%). The patients were treated by a consistent policy of surgical removal with histological verification followed by radiation therapy with or without chemotherapy. The 10- and 20-year survival rates of patients with pure germinoma were 92.7% and 80.6%, respectively. The 10-year survival rates of patients with mature teratoma and malignant teratoma were 92.9% and 70.7%, respectively. Patients with pure malignant germ cell tumors (embryonal carcinoma, yolk sac tumor, or choriocarcinoma) had a 3-year survival rate of 27.3%. The mixed tumors were divided into three subgroups: 1) mixed germinoma and teratoma; 2) mixed tumors whose predominant characteristics were germinoma or teratoma combined with some elements of pure malignant tumors; and 3) mixed tumors with predominantly pure malignant elements. The 3-year survival rates were 94.1% for the first group, 70% for the second group, and 9.3% for the third group, and the differences were statistically significant. Twenty-six patients with malignant tumors received chemotherapy that consisted of cisplatin and carboplatin combinations with or without radiation therapy. However, chemotherapy was not significantly more effective than radiation therapy alone. From these treatment results, the authors classified tumors into three groups with different prognoses and proposed a treatment guideline appropriate for the subgroups.     

Unilateral lobectomy for Hurthle cell adenoma

Considerable controversy exists regarding the ability to predict the biologic behavior of Hurthle cell tumors. Some have found the clinicopathologic criteria used to differentiate benign from malignant lesions to be unreliable and have advocated total thyroidectomy for all Hurthle cell neoplasms. From January 1980 to December 1995, 39 patients had surgery for Hurthle cell tumors of the thyroid. The surgical pathologic findings were reviewed by an experienced pathologist (JP). Eight patients had histologic findings of capsular or vascular invasion consistent with carcinoma and had total thyroidectomy. Four of these patients had postoperative evidence of residual disease and were treated by radiation ablation. No evidence of invasion was found in 31 patients diagnosed with Hurthle cell adenoma. Twenty-three of these patients had unilateral lobectomy; total thyroidectomy was done in the remaining 8 patients, 5 of whom were found to have an associated papillary carcinoma at the time of operation. There were no operative deaths or significant morbidity. Twenty-two adenomas (71%) were found in females, whereas males had malignant tumors in 6 of 8 cases (P = 0.025). The mean age of adenoma patients is 54.1 years, and that of the carcinoma patients is 55.8 years. Mean size of benign tumors was 2.8 cm and of malignant tumors 4.1 cm (P = 0.04). Four of seven (57%) carcinomas were larger than 4 cm as compared with 6 of 30 (20%) adenomas (P = 0.069). Follow-up has ranged from 1 month to 15 years, with a mean of 3.2 years. There have been no deaths, and no patients with Hurthle cell adenoma have had evidence of recurrence or metastases during follow-up. Our data suggest that carcinoma patients tend to be male and tumor size is larger. An association was found when trying to predict malignancy by using 4 cm as a threshold size. We conclude that pathologic evidence of capsular or angioinvasion can accurately differentiate benign from malignant tumors. Unilateral thyroid lobectomy is adequate therapy for the treatment of Hurthle cell adenoma, with total thyroidectomy reserved for those patients with histologically proven carcinoma.     

Mediastinal teratomas: review of 15 pediatric cases

One hundred fifty-three children with a teratoma presented to one hospital between 1970 and March 1992. The clinical and pathological features of 15 patients with mediastinal teratomas are reviewed; six were newborn and nine aged from infancy to 13 years. Thirteen patients including the six newborns presented with respiratory distress and all 15 patients had a mass on chest radiograph. A definite diagnosis of teratoma was not made preoperatively in any of these patients. At operation, a median sternotomy was used to approach seven anterior tumors and a lateral thoracotomy performed in the other eight patients. Histologically two were mature, 10 had immature elements, and three were malignant teratomas. The patients with malignant tumors were all over 12 years of age and died within 6 months of treatment. All six neonates had immature teratomas. Raised serum alpha-fetoprotein levels provided useful markers in two patients with recurrent tumors. Three conclusions can be drawn: (1) mediastinal teratomas are rare in children and frequently are not diagnosed before operation; (2) in newborns these tumors may be immature and present with respiratory distress; and (3) a median sternotomy gives excellent exposure for anterior mediastinal tumors.     

Concomitant infusion cisplatin and hyperfractionated radiotherapy for locally advanced nasopharyngeal and paranasal sinus tumors

PURPOSE: This is a prospective study to improve the therapeutic ratio in the treatment of patients with locally advanced nasopharyngeal and paranasal sinus tumors by using split-course concomitant infusion cisplatin chemotherapy and hyperfractionated radiotherapy. METHODS AND MATERIALS: From 1983 to 1993, 21 patients with locally advanced nasopharyngeal and paranasal sinus tumors (T3 and T4, or recurrent tumors involving the facial bones and/or the base of the skull) were treated with a regimen of split-course hyperfractioned radiotherapy (1.2 Gy/fraction/bid) and concomitant infusion cisplatin (5-10 mg/m2/24 h). The therapy was given in three separate 2-week sessions with 1 to 2 week breaks between sessions. Seventeen of 21 patients were treated with curative intent with cumulative radiation doses ranging from 64.8 to 70.8 Gy. Four patients were treated with palliative intent to a total dose of less than 60 Gy or to a limited field due to previous irradiation. RESULTS: Sixteen of 17 patients (94%) treated curatively achieved a complete response. Of the 16 patients who achieved complete response, 7 patients (50%) were alive at the time of analysis (36 to 126 months). One patient was alive at 4 years with no evidence of disease, and died in 10 years at the age of 80 of unknown cause. Two patients died of local recurrence at 21 and 45 months and one patient died of a cerebrovascular accident at 12 months with disease status unknown. Five patients died of distant metastases. The one patient who had a partial response died in 25 months with local disease and metastases to the bone and lung. Four patients that were previously irradiated received a reduced total dose or treated to a limited irradiation field. All had near complete responses, but died within a year of treatment, with the exception of one patient who died at 23 months. Acute reactions included intense erythema of the mucosa in all patients. Five of 21 (23%) developed punctate mucositis and 3 of 21 (14%) developed confluent mucositis. Hematologically, one patient developed neutropenia (1800 WBC/mm3) and one developed thrombocytopenia (38,000/mm3). A rising creatinine was observed in three patients (2.0, 1.7, 1.7) all of whom were treated with the higher 10 mg/m2/day dose of infusional cisplatin. In all three of these cases, the creatinine slowly returned to normal over a 6-month period. Hormonal evaluations were performed in three patients and all were within normal ranges. There was no evidence of neck fibrosis or trismus. One patient with gross recurrent disease of the orbit developed blindness of the involved eye due to corneal opacification. The orbital area had been reirradiated in this patient. CONCLUSIONS: Concomitant infusion cisplatinum with hyperfractionated radiation improved tumor control, but did not increase normal tissue injury. Acute reactions were minimized by splitting the treatment with a 1- to 2-week break after each 2 weeks of radiation treatment. Late complications were not increased by using a hyperfractionated radiation regimen. The local failure rate was only 18% (3 of 17 patients), but the distant failure rate was 35% (6 patients). Further investigation is needed to prove if adjuvant chemotherapy after concomitant chemoradiation improves survival by decreasing the distant failure in such advanced cases.     

Heat shock alters poly(A) tail length of Dictyostelium discoideum hsp32 RNA

BACKGROUND: Primary leiomyosarcoma of bone is a very rare malignant tumor with uncertain pathogenicity. METHODS: The authors studied five cases of surgically treated primary leiomyosarcoma of bone. Clinical histories and radiographic findings were recorded. Regular clinical and radiographic controls were obtained postoperatively. In all cases, immunohistochemical studies were used to confirm the diagnosis. Molecular biologic examinations, using the polymerase chain reaction technique with microsatellite DNA markers from regions of tumor-relevant genes, were performed to determine the stability of the genome or to detect some typical genomic changes. RESULTS: The study included three women and two men, with an average age of 42 years. The tumor was located in the pelvis in two patients, in the femur in two patients, and in the proximal tibia in one patient. All tumors were classified as high-grade tumors (four stage IIB, one stage IIA). Radiographically, all tumors appear as purely osteolytic lesions, with a geographic or moth-eaten appearance and without any sclerotic margin. Three patients underwent limb salvage surgery followed by endoprosthetic replacement. The other two patients required amputation. The mean follow-up was 19 months (range, 8-29 months). Three patients died of disease, with a mean postoperative survival period of 18 months (range, 6-27 months). Four patients developed diffuse pulmonary metastases after an average of 10.5 months. One of those patients responded well to chemotherapy. In all cases, immunohistochemistry showed strong reactivity of the tumor cells for (alpha-SMA and vimentin. Molecular biologic investigations revealed a high rate of genomic instabilities in all of the stage IIB tumors. CONCLUSION: Clinical follow-up suggests that primary osseous leiomyosarcoma has an aggressive biologic behavior. The immunohistochemical studies are useful tools and suggest that osseous leiomyosarcoma arise from the vascular smooth muscle cells within the bone. The molecular biologic findings of a high rate of genomic instability confirm the hypothesis that this rare entity is of an aggressive nature.     

Hürthle cell tumors

OBJECTIVES: a) To provide a clinicopathological profile of Hürthle cell neoplasms (HCT) in our experience. b) To evaluate if there are any differences in the clinical or morphological features between three HCT categories: benign, malignant and indeterminate. c) To examine the role of the clinical and morphological features in predicting the behavior of these neoplasms. METHODS: We reviewed the clinical reports of all patients with a histological diagnosis of HCT at our Hospital between 1981 and 1996. The final study group consisted of 25 cases. The neoplasms were divided into three categories on the basis of presence and degree of capsular and vascular invasion, marked nuclear atypia, tumour necrosis and pattern of growth. A series of clinical parameters were evaluated. RESULTS: Of the 25 tumors, 52% were morphologically classified as benign, 8% as indeterminate and 40% as malignant. Follow-up ranged from 10 months to 14.8 years or until death (average 3.8 years). There were four local recurrences (20%), three in the malignant group (30%) and one in the benign group (7.6%) (p = 0.15). One patient presented metastases and died because of tumor during the follow-up. Apart from capsular and vascular invasion and some aspects of therapy, no significant differences were found in the clinical and histological parameters analyzed between the three histological groups or between the groups with or without recurrence. CONCLUSION: We did not find any clinical or morphological parameter which can predict recurrence among these tumors. Our study further establishes the controversial issues surrounding the biological behavior of Hürthle cell neoplasms.     

The management of tumors of the ampulla of Vater by local resection

OBJECTIVE: The authors report on indications and results of local excision of tumors of the ampulla of Vater. SUMMARY BACKGROUND DATA: Local excision of ampullary tumors has been performed for nearly a century but remains controversial. The use of this procedure for benign conditions is clear, but its place, if any, in the management of ampullary malignancy is debated. METHODS: The presentation, evaluation, and treatment of 26 patients who underwent local resection of ampullary tumors between January 1987 and November 1994 are reviewed. RESULTS: There were 16 men and 10 women, with a median age of 58 years. Eighteen patients had adenomas, whereas 8 patients had adenocarcinomas. Patients presented predominantly with jaundice (50%), pain (35%), and pancreatitis (27%) and were evaluated with endoscopic retrograde cholangiopancreatography and biopsy. All patients with benign lesions had accurate preoperative biopsies. Two of eight patients shown intraoperatively to have malignant lesions had preoperative biopsies read as benign. There were no deaths. Postoperative complications included two wound infections and one episode each of cholangitis, lower gastrointestinal bleeding, and adhesive gastrointestinal obstruction. All patients had prompt resolution of jaundice if present before surgery, and the mean postoperative stay was 7.5 days. Six of eight patients with malignant lesions have had recurrent disease. CONCLUSIONS: Local excision of malignant ampullary tumors is effective palliative therapy when the patient is unfit for the Whipple procedure. Ampullary resection usually is curative for patients with benign lesions without a polyposis syndrome. In this series, intraoperative frozen section routinely was accurate.     

Sertoli cell tumors of the testis, not otherwise specified: a clinicopathologic analysis of 60 cases

Sixty Sertoli cell tumors of the testis, excluding large cell calcifying and sclerosing subtypes, are described. Patient age ranged from 15 to 80 years (mean, 45 years). The initial manifestation was usually a testicular mass; in 14 cases it had been enlarging slowly for a period of up to 14 years (mean 3.7 years). Only five patients had testicular pain. Four patients had metastatic disease at the time of presentation. All the tumors were unilateral and ranged from 0.3 cm to 15 cm (mean 3.6 cm). They were typically well circumscribed. Sectioning usually disclosed firm, tan-gray, white, or yellow tissue with areas of hemorrhage and a minor cystic component in approximately one third. Microscopic evaluation usually revealed diffuse sheets or large, nodular aggregates of tumor cells, within which solid or hollow, sometimes dilated, tubules and, less often, cords were usually at least focally identifiable. A relatively acellular, often vascular, fibrous to hyalinized stroma was frequently conspicuous. The tumor cells typically had moderate amounts of pale to lightly eosinophilic cytoplasm, but 10 tumors had cells with abundant eosinophilic cytoplasm. Large cytoplasmic vacuoles were prominent in 26 tumors. Nuclear atypicality was absent or mild in 54 cases, moderate in 4 cases, and marked in 2 cases. Mitotic rate ranged from less than 1 to 21 per 10 high power fields, with 50 tumors having no or only rare mitoses. Vascular space invasion was present in 11 cases and was prominent in 8. Follow-up of more than five years (average 8.4 years), or until evidence of metastasis was seen, was available for 16 patients. Nine were alive and well with no evidence of disease. Four were alive with disease and three died of disease. The pathologic features that best correlated with a clinically malignant course were as follows: a tumor diameter of 5.0 cm or greater, necrosis, moderate to severe nuclear atypia, vascular invasion and a mitotic rate of more than 5 mitoses per 10 high power fields. Only one of nine benign tumors for which follow-up data of 5 years or more were available had more than one of these features, whereas five of seven malignant tumors had at least three.