Meningiomas of the jugular foramen

Primary meningiomas of the jugular foramen are extremely rare. There is controversy regarding the management of these tumors. The objective of this article is to analyze the treatment and results in a series of ten cases. From a series of 107 patients that had been operated on for jugular foramen tumors between 1987 and 2005, ten had meningiomas. The clinical, histological and imaging findings, as well as the outcome of these cases, were reviewed. A high incidence of malignant or aggressive tumors (six cases) was found. Gross total resection (Simpson I and II) was achieved in five patients. Four patients with meningotheliomatous meningiomas are alive, with a mean follow-up time of 71.8 months (6.5 years). Two patients (one with anaplastic type and one with papillary type) died in the immediate postoperative period. Four patients (two with papillary type, one with microcystic type and one with anaplastic type) died because of disease progression, with a mean survival time of 35 months. Radical removal of benign jugular foramen meningiomas is possible. The incidence of postoperative deficit of cranial nerves is higher than in other benign tumors of the jugular foramen. A high incidence of aggressive (malignant) tumors was observed in this series.     

Giant lipomatous tumors of the upper extremities--a series of 8 consecutive cases

We present a series of eight consecutive patients evaluated in our department from 2002 to 2007 with giant lipomatous tumors (as defined in medical studies as greater than 5 cm) of upper extremities. There were three male and five female, ranged in age from 17 to 77 years (mean age of 55). The tumor's size ranged from 5 to 34 cm. All patients underwent total excision of the tumors with free margins. All specimens were sent to pathology: seven patients had benign tumors and one patient had liposarcoma. There were no recurrences of the tumors. Appropriate preoperative evaluation and complete surgical excision are mandatory for successful treatment of these tumors.     

Primary Malignant Mediastinal Tumors

Thirty-eight patients with primary malignant mediastinal tumors of all cell types are the basis for this review. Eleven of these patients had germ cell tumors. Five germ cell tumors were seminomas, two were malignant teratomas, and two were endodermal sinus tumors. Mean survival for all patients with germ cell tumors was 3.3 years. Eight children had surgical excision of mediastinal neuroblastomas, and all but 1 are alive for a mean survival of 6.7 years. Seven patients had lymphoproliferative disorders; 6 of these patients had nodular sclerosing Hodgkin's disease, and 1 had lymphoblastic (thymic) lymphoma. Mean survival was 5.1 years. There were five carcinomas of various cell types and one angiopericytoma. None of the patients with these lesions survived more than 2 years. Four patients had thymoma with an average survival of 3.7 years. Two patients had carcinoid tumors of thymic origin; neither survived more than 1 year.In 1972, we reported 5-year disease-free survival of 26% in a series of patients with primary mediastinal tumors. Our experience since 1970 shows current survival of 47.3% and 5-year disease-free survival of 34.2%. We use combined methods of therapy, including aggressive surgical resection, combination chemotherapy, and often mediastinal irradiation for most types of mediastinal tumors. Primary mediastinal malignancies should be treated aggressively using a multidisciplinary approach, since many of these tumors are curable.     

Resection of pancreatic neuroendocrine tumors: results of 70 cases

HYPOTHESIS: Neuroendocrine tumors of the pancreas can be managed surgically with excellent outcomes. DESIGN: Retrospective case series. SETTING: Academic medical center. PATIENTS: Seventy consecutive patients who underwent resection for pancreatic neuroendocrine tumors between January 1, 1990, and December 31, 2005. INTERVENTIONS: Pancreaticoduodenectomy, distal pancreatectomy, or enucleation. MAIN OUTCOME MEASURES: Postoperative morbidity, mortality, and long-term survival. RESULTS: Of the 70 patients, 50 (71.4%) had nonfunctional tumors. Thirty-seven patients (52.9%) had neuroendocrine carcinomas and 13 (18.6%) had benign islet cell neoplasms. Twenty patients had functional tumors. Of these 20 patients, 16 had insulinomas, 2 had glucagonomas, and 2 had gastrinomas. Twenty-seven patients underwent pancreaticoduodenectomy, 32 had distal pancreatectomy, and 11 underwent enucleation. Patients undergoing enucleation as compared with those not undergoing enucleation were younger (mean age, 39 vs 51 years, respectively; P = .009) and had smaller tumors (mean tumor size, 2 vs 5 cm, respectively; P<.001). Postoperative complications occurred in 13 patients (48.1%) after pancreaticoduodenectomy, in 4 patients (12.5%) after distal pancreatectomy, and in 0 patients after enucleation. There were no perioperative mortalities. With a median follow-up of 50 months, the 5-year actuarial survival for the patients with malignant neuroendocrine carcinomas (n = 37) was 77%, and all of the patients with functional tumors are alive. The presence of lymphovascular invasion closely approached significance when survival was evaluated (P = .06). Lymph node status, perineural invasion, and liver metastasis did not impact survival. CONCLUSIONS: This single-institutional case series demonstrates that pancreatic neuroendocrine tumors can be safely resected without mortality and with minimal morbidity. The presence of lymphovascular invasion can be used to classify neuroendocrine tumors as malignant, and this appears to predict survival. Patients with malignant tumors can expect long-term survival even in the setting of metastatic disease.     

Flow cytometric analysis of DNA content as a prognostic indicator in squamous cell carcinoma of the tongue

The mortality of squamous cell carcinoma of the tongue has not significantly improved in decades. Much of the information that has been gathered to date has been based on retrospective analyses. There is little consensus on treatment of the disease. In an attempt to define an objective prognostic indicator of aggressiveness of these tumors, a retrospective analysis of 15 paraffin-embedded specimens using flow cytometry was performed. Ten patients (67 percent) had aneuploid tumors and had a 5 year disease-free survival rate of 33 percent, whereas patients with diploid tumors (33 percent) had a 5 year disease-free survival rate of 80 percent. Although the number of patients was small, it appears that flow cytometry may be an objective prognostic indicator in patients with squamous cell carcinoma of the tongue. Larger series of archival paraffin-embedded flow cytometry analyses are recommended, as well as examination of variables other than the disease-free survival rate.     

Transitional cell carcinoma of bladder in patients under thirty years of age.

Seventy-nine cases of transitional cell carcinoma of the bladder in the age group under thirty years have been reviewed. The diagnosis and treatment are the same as in the older age group. The tumors are usually of low grade and low stage, but they can become more aggressive and more malignant. Contrary to previously held beliefs that transitional cell tumors in the younger age group rarely if ever recur, this series had a recurrence rate of 13.9 per cent with several patients having multiple recurrences. Therefore, the follow-up care of these patients must be vigilant and persistent.     

Gastrointestinal carcinoid tumors

Fifty-five patients were evaluated and treated for gastrointestinal (GI) carcinoid tumors at our institution from 1968 through 1988. Thirty patients had primary tumors smaller than 1 cm and were treated surgically or endoscopically by local excision, whereas 25 underwent resection with regional lymphadenectomy for larger primaries. At the time of initial treatment 17 patients demonstrated metastasis. Although 13 of these patients had primary tumors larger than 2 cm, in four patients with ileal carcinoids, the primary measured 0.8 to 1.5 cm. Serosal involvement was present in nine of 17 patients with metastasis. With a follow-up range of 3 months to 20 years, six patients have died with metastatic carcinoid tumor, two patients died without evidence of disease, 12 patients are alive with metastasis, and 35 patients are alive, free of disease. In this series three determinants for risk of metastasis in GI carcinoid tumors were evident: primary size, serosal penetration, and primary site in the small intestine. In patients with any of these risk factors, resection with regional lymphadenectomy is recommended.     

Ovarian epithelial tumors of low malignant potential: a case series of 5 adolescent patients

Epithelial ovarian neoplasms are uncommon in pediatric and adolescent patients, accounting for approximately 20% to 30% of ovarian tumors in adolescent females and women younger than 25. Tumors of low malignant potential (LMP) account for a significant proportion of epithelial neoplasms in this patient population. This case series describes 5 adolescent patients, with a mean age of 14.4 ± 2.4 years, diagnosed with ovarian tumors of LMP at one institution.Between November 2001 and January 2006, 5 patients were diagnosed with ovarian tumors of LMP of 126 patients who had surgery for adnexal masses. All patients underwent initial surgery via laparotomy. Two patients underwent ovarian cystectomy, and 3 had at least a unilateral salpingo-oophorectomy. One patient had stage IIIc disease, whereas the other 4 patients, not all completely staged, had presumed stage I disease. Three patients developed recurrent ovarian masses on follow-up. Two had recurrent LMP tumors (one bilateral) and one was a benign mucinous cystadenoma.This case series of 5 adolescent patients with ovarian tumors of LMP highlights the importance of considering epithelial neoplasms in any pediatric or adolescent patient with a pelvic mass and supports conservative management, with staging and fertility-sparing surgery; however, appropriate follow-up is essential, as evidenced by 3 of 5 patients exhibiting recurrent ovarian masses.     

Long-term results after radiosurgery for benign intracranial tumors

BACKGROUND: Stereotactic radiosurgery is the principal therapeutic alternative to resecting benign intracranial tumors. The goals of radiosurgery are the long-term prevention of tumor growth, the maintenance of patient function, and the prevention of new neurological deficits or adverse radiation effects. Evaluation of long-term outcomes more than 10 years after radiosurgery is needed. METHODS: We evaluated 285 consecutive patients who underwent radiosurgery for benign intracranial tumors between 1987 and 1992. Serial imaging studies were obtained, and clinical evaluations were performed. Our series included 157 patients with vestibular schwannomas, 85 patients with meningiomas, 28 patients with pituitary adenomas, 10 patients with other cranial nerve schwannomas, and 5 patients with craniopharyngiomas. Prior surgical resection had been performed in 44% of these patients, and prior radiotherapy had been administered in 5%. The median follow-up period was 10 years. RESULTS: Overall, 95% of the 285 patients in this series had imaging-defined local tumor control (63% had tumor regression, and 32% had no further tumor growth). The actuarial tumor control rate at 15 years was 93.7%. In 5% of the patients, delayed tumor growth was identified. Resection was performed after radiosurgery in 13 patients (5%). No patient developed a radiation-induced tumor. Eighty-one percent of the patients were still alive at the time of this analysis. Normal facial nerve function was maintained in 95% of patients who had normal function before undergoing treatment for acoustic neuromas. CONCLUSION: Stereotactic radiosurgery provided high rates of tumor growth control, often with tumor regression, and low morbidity rates in patients with benign intracranial tumors when evaluated over the long term. This study supports radiosurgery as a reliable alternative to surgical resection for selected patients with benign intracranial tumors.     

Survival of patients with well-differentiated astrocytomas diagnosed in the era of computed tomography

We report 25 verified cases of well-differentiated cerebral astrocytomas in adults treated between 1978 and 1988. All patients were diagnosed by computed tomographic (CT) scans, with 5 undergoing a craniotomy for debulking and 20 undergoing a biopsy alone. The median survival for the entire group was 8.2 years, the longest survival yet reported for a series of patients with these tumors. A review of the literature suggests that the longer survival observed in more recent series is the result of the earlier diagnosis of tumors afforded by modern brain imaging. Twenty of our patients presented with seizures in the absence of any other focal findings and would probably not have had a biopsy in the era before CT scans until their tumors had progressed. Only 8% of our patients had papilledema at the time of presentation, in contrast to almost half of the patients with low-grade astrocytomas reported before 1975, supporting the hypothesis that patients in the CT era are diagnosed earlier. None of our patients died from progressive low-grade disease. One patient died from a squamous cell cancer, and 7 died as a consequence of their tumors dedifferentiating into a more malignant astrocytoma or glioblastoma multiforme, with a median time of approximately 5 years after the diagnosis. Our findings, together with the available data in the literature, suggest that death from a focal low-grade astrocytoma, in the absence of malignant degeneration, may be a rare event. Consequently, future therapeutic efforts should be targeted at preventing dedifferentiation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Association of urinary bladder paragangliomas with germline mutations in the SDHB and VHL genes

ObjectiveOur primary goal was to examine the clinical characteristics of a series of patients with urinary bladder paragangliomas (UBPGLs), focusing particularly on their genetic backgrounds.Materials and methodsWe analyzed the medical records of patients who presented to the National Institutes of Health with UBPGL from 2000 to 2013 to determine their clinical characteristics and outcomes, biochemical phenotype, tumor size, and genetic background.ResultsOf the 27 patients with UBPGLs who were identified, 17 (63%) had underlying genetic mutations. Overall, 14 (51.9%) patients had a germline mutation in the succinate dehydrogenase subunit B gene (SDHB), and 3 (11.1%) had mutations in the von Hippel-Lindau gene (VHL). Of the 21 patients who had biochemical data available before their first operation, 19 (90.5%) presented with a noradrenergic biochemical phenotype; 7 (33.3%) patients had tumors that also secreted dopamine. In addition, 1 patient (4.8%) had elevated metanephrine levels, and 2 (9.5%) had normal biochemical data. In total, 13 (48.1%) patients in the series were diagnosed with metastatic disease, at either first presentation or follow-up; 6 of these patients (46.1%) had SDHB mutations.ConclusionsUBPGLs typically present with a noradrenergic phenotype and are frequently associated with underlying germline mutations. Patients presenting with these rare neuroendocrine tumors should be screened for these mutations. In addition, patients with UBPGLs should be followed up closely for metastatic development regardless of genetic background, as almost half of the patients in this series presented with metastatic disease and less than half of them had SDHB mutations.     

Estrogen and progesterone receptors in meningiomas

Two-thirds of all meningiomas and four-fifths of intraspinal and sphenoidal meningiomas occur in women. Meningiomas frequently enlarge or become symptomatic during pregnancy or during the luteal phase of the menstrual cycle. There is an increased incidence of meningiomas in women with breast carcinoma. In a series of 23 patients with meningiomas, the authors assayed biopsy specimens of the tumor for the presence of estrogen (ER) and progesterone (PR) receptors, using glycerol density gradient centrifugation and dextran-coated charcoal techniques. Significant levels of ER were found in only 17% of the patients, while significant PR levels were detected in 39%. Only one of the 16 tumors from female patients had significant ER levels, whereas three of the seven tumors from men had significant ER levels. Eight of the 16 tumors in women had significant PR levels, whereas only one of the seven tumors in men had a significant PR level. Thus, three out of four tumors with definite ER were from men, whereas eight of nine tumors with definite PR were from women. Of the eight women whose tumors contained PR, three were premenopausal and five postmenopausal. The single tumor with high levels of PR in the male patient was histologically atypical. The results of this series were compared with six published series of sex steroid assays in meningiomas. These seven series were divided into two groups: one group included two reports from the same laboratories in France, and the other the remaining five reports. Much higher percentages of both ER- and PR-positive tumors were reported from the French group. The authors suggest that this discrepancy may be due to the use of preoperative glucocorticoid therapy in the series from the United States. Since meningiomas are known to enlarge during periods when levels of circulating progestins are high, the presence of significant quantities of PR in a high percentage of tumors may have therapeutic implications for recurrent, malignant, or incompletely excised tumors, or for medically fragile patients. Conversely, since meningiomas are not known to enlarge during the proliferative phase of the menstrual cycle or with exogenous estrogen therapy, the small number of tumors positive for ER may indicate that ER lacks clinical significance. High levels of PR found in a small group of histologically aggressive tumors in several series may indicate that hormonal therapy may be especially useful in this difficult subset of patients.     

Gastrointestinal carcinoids: characterization by site of origin and hormone production

OBJECTIVE: To describe a large series of patients with carcinoid tumors in terms of presenting symptoms, hormonal data, stage at diagnosis, pathologic features, and survival. SUMMARY BACKGROUND DATA: Published series have described significant prognostic features of carcinoid tumors as site of origin, age, sex, stage at diagnosis, presence of high hormone levels, and increased T stage. Of these, stage at diagnosis and T stage seem to emerge most often as independent predictors of survival in multivariate analyses. Of carcinoid tumors, those arising from a midgut location have higher levels of serotonin and serotonin breakdown products, as well as more frequent metastatic disease at presentation, than those arising from either foregut or hindgut locations. METHODS: A prospective database of carcinoid patients seen at Duke University Medical Center was kept from 1970 to the present. Retrospective medical record review was performed on this database to record presenting symptoms, hormonal data, pathologic features, and survival. Statistical methods included analysis of variance, Kaplan-Meier analysis, and Mantel-Cox proportional hazard survival analysis, with P <.05 considered significant for all tests. RESULTS: Carcinoids arising in different locations had different presentations: rectal carcinoids presented significantly more often with gastrointestinal bleeding, and midgut carcinoids presented significantly more often with flushing, diarrhea, and the carcinoid syndrome. Patients with midgut tumors had significantly higher levels of serotonin and serotonin breakdown products, corresponding to higher metastatic tumor burdens. Although age, stage, region of origin, and urinary level of 5-hydroxyindoleacetic acid predicted survival by univariate analysis, only the latter three were independent predictors of survival by multivariate analysis. Of the patients with metastatic disease at diagnosis, those with midgut tumors had better survival than those with foregut or hindgut tumors. CONCLUSIONS: Although region of origin is certainly an important factor in determination of prognosis, stage of disease at presentation is more predictive of survival. Pancreatic and midgut carcinoids are metastatic at diagnosis more often than those arising in other locations, leading to a worse overall prognosis. Among patients with distant metastases, patients with midgut primary tumors have improved survival despite increased hormone production compared with patients with tumors arising in other primary sites.     

Primary small bowel malignant tumors. Unrecognized until emergent laparotomy

Forty-five consecutive cases of primary small bowel malignant tumors treated at our institution from 1969-1983 were reviewed. Sixty-four per cent of the patients had surgical emergencies. Fourteen patients had intestinal obstruction, 11 had gastrointestinal (GI) hemorrhage, and 4 had perforation of the small bowel. The remaining 16 patients were explored for persistent symptoms, an abnormality on GI series, or a mass lesion on CT scan. Thirty-eight patients underwent resection of the tumor and seven had palliative bypass procedures. This study suggests that a high index of suspicion and early diagnostic evaluation including a small bowel series is necessary to prevent the large number of small bowel tumors presenting as surgical emergencies. In particular, small bowel tumors should be suspected in patients with abdominal pain of unknown etiology, unexplained weight loss, or occult GI bleeding.     

Management of Carotid Body Paragangliomas and Review of a 30-Year Experience

Carotid body tumors are rare neoplasms and must be considered in the evaluation of all lateral neck masses; early surgical removal has been recommended. In this study, the medical records of 29 patients with 34 carotid body paragangliomas who were treated at our institution between 1971 and 2001 were retrospectively reviewed. An overview is provided of this lesion, including diagnosis, classification, metastatic potential, possible secretory function, operative techniques, and nonsurgical methods of management. Carotid body tumors may be familial and are more often bilateral in these instances; five patients (17%) had bilateral tumors in this series. The criterion for malignancy is demonstrated by metastatic tumor in lymph nodes or distant organs. Three patients (10%) had malignant tumors, one with hepatic metastases. One patient (3%) in our series exhibited abnormal serotonin production. Vascular reconstruction was necessary in eight cases (28%). No stroke occurred, however, two arterial thromboses (7%), five permanent cranial nerve deficits (17%), and one death (3%) from massive pulmonary embolism were seen. Our experience demonstrates that early operative management is warranted to avoid the possibility of eventual metastasis and progressive local invasion to the point of inoperability.     

Neoplasms of the Small Bowel

Small bowel tumors are unusual lesions exhibiting nonspecific clinical features often diagnosed at an advanced stage. In the cases studied at the Hospital of the University of Pennsylvania nearly all the 32 patients with malignancies were symptomatic whereas in the 34 patients with benign lesions the condition was discovered as an incidental finding in about half of the patients. Weight loss, palpable mass or anemia usually indicated malignancy. Small bowel radiography was the most useful diagnostic aid in the present series. While the etiology of these lesions is unknown, villous adenomas probably bear a relationship to carcinoma. The association between chronic regional enteritis and small bowel tumors is unestablished but suggestive. An analysis of reported series reveals a disproportionate incidence of additional primary tumors in patients with small bowel neoplasms. Surgical extirpation is indicated for curative treatment. In the present series, resection in hope of cure was carried out in 25 of 32 malignant tumors resulting in eight five-year survivals. One of these latter lived nine years with disseminated malignant carcinoid reflecting the occasional indolent course of this tumor.     

A 20-year review of pediatric pancreatic tumors.

Pancreatic tumors are rare surgical problems in infants and children. A 20-year audit (1971 to 1991) of this institution showed six patients ranging in age from 3 weeks to 16 years who were operated on for pancreatic neoplasms. Five of these tumors were malignant, bringing the reported experience to 71 cases. This series of malignancies included three solid cystic tumors, one insulin-secreting tumor, and one pancreatoblastoma. The clinical presentations varied: three had abdominal pain, one developed hypoglycemia, and one had an abdominal mass with jaundice. In five of the six patients pancreatic pathology was suspected preoperatively. All were treated primarily with pancreatic resection including one pancreatoduodenectomy. No radiotherapy or chemotherapy was used. The perioperative mortality was 0% with a morbidity of 50%. The long-term results are encouraging, with all patients alive after a mean follow-up of 7.8 years. These data suggest that aggressive surgical therapy is warranted in the management of pediatric pancreatic tumors.     

Primary facial nerve tumors within the skull

In a series of 527 cerebellopontine angle tumors, there were 416 cases of acoustic nerve tumors and 14 cases of primary tumor of the facial nerve in the petrous bone or intracranial cavity. Six additional patients were presumed to have facial tumors, although they were not operated on. Of the 14 verified facial nerve tumors, all but two were neurinomas and 11 had important intracranial extensions into the middle and/or the posterior fossa. In most of these 14 cases, surgical removal was performed via the translabyrinthine route, which is advantageous in that it displays the characteristic relationship of the tumor to the facial nerve, and facilitates nerve repair. The clinical and radiological features of these facial nerve lesions are discussed and also the indications for surgical treatment which, as the unoperated cases illustrate, is not always necessary.     

Vagal paraganglioma.

OBJECTIVES: To report the outcomes of surgical treatment of vagal paragangliomas and to define a management protocol. DESIGN: A retrospective case series. PATIENTS AND METHODS: Sixteen consecutive patients with vagal paragangliomas managed by surgical resection using a cervicoparotid approach and pericapsular dissection using microsurgical techniques between 1990 and 2003. RESULTS: All patients either had or developed a vagal palsy. Additional cranial nerve deficits were sustained in 8 patients. No patients died as a result of surgery or from their disease. CONCLUSIONS: The technique used and described in this article allowed adequate exposure of the retrostyloid parapharyngeal space for the safe removal of all vagal tumors in this series. Careful consideration must be given to the likely natural progression of these tumors before committing to surgical resection. This is particularly important in patients with multifocal disease.