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Nancy D Perrier

Publications and source records attributed to Nancy D Perrier.

At least 19 recordsLinked to original sources
PubMed2007

Primary adrenal natural killer/T-cell nasal type lymphoma: first case report in adults.

We report the first case of a primary adrenal natural killer (NK)/T-cell nasal type lymphoma in adults. The patient presented with an enlarging left adrenal mass and the initial concern was for adrenocortical carcinoma. Surgical resection revealed NK/T-cell lymphoma. Rapid recurrence in the contralateral adrenal gland was treated with a single cycle of chemotherapy before he died due to infectious complications and progressive disease. This case demonstrates the aggressive presentation of a novel subset of primary adrenal lymphoma that should be considered in the differential diagnosis of a rapidly enlarging adrenal mass.

Adrenal Gland Neoplasms↗
PubMed2006

Improved preoperative planning for directed parathyroidectomy with 4-dimensional computed tomography.

BACKGROUND: Four-dimensional computed tomography (4D-CT) provides both functional and highly detailed anatomic information about parathyroid tumors. The purpose of this study was to compare 4D-CT with sestamibi imaging and ultrasonography as methods for the accurate preoperative localization of hyperfunctioning parathyroid glands before parathyroidectomy. METHODS: A study of 75 patients with primary hyperparathyroidism was performed at a tertiary-care institution. Sestamibi imaging, ultrasonography, and 4D-CT were performed on each patient preoperatively. Results of the imaging studies were compared with operative findings, pathologic data, and biochemical measurements to assess the sensitivity and specificity of each of the imaging modalities. RESULTS: 4D-CT demonstrated improved sensitivity (88%) over sestamibi imaging (65%) and ultrasonography (57%), when the imaging studies were used to localize (lateralize) hyperfunctioning parathyroid glands to 1 side of the neck. Moreover, when used to localize parathyroid tumors to the correct quadrant of the neck (ie, right inferior, right superior, left inferior, or left superior), the sensitivity of 4D-CT (70%) was significantly higher than sestamibi imaging (33%) and ultrasonography (29%). CONCLUSION: 4D-CT provides significantly greater sensitivity than sestamibi imaging and ultrasonography for precise (quadrant) localization of hyperfunctioning parathyroid glands. This allows improved preoperative planning, particularly for the case of reoperation. In addition to the data that are provided, we present a novel classification scheme for use in parathyroid localization.

Adult↗
PubMed2006

Vacuum-assisted closure for the treatment of complex breast wounds.

Complex breast wounds are a constant problem for surgeons. Wound vacuum-assisted closure therapy (VAC) has been shown to be effective for a variety of complex wounds. Our goal was to evaluate our experience with the (VAC) device in the treatment of open breast wounds. We retrospectively identified 18 patients with complex breast wounds treated with the VAC. We analyzed the data regarding the nature and management of these wounds using the VAC device. Fifteen of 18 patients were treated effectively using the VAC. Two patients required muscle flap coverage. One patient had the VAC dressing discontinued secondary to a denial by an insurance company for VAC in the home setting. VAC therapy is an effective treatment for complex wounds. Specifically, our experience shows it to be effective in the treatment of complex breast wounds. Utilization of VAC therapy should be considered for the management of these challenging wounds.

Breast Neoplasms↗
PubMed2006

Abdominal visceral lesions in von Hippel-Lindau disease: incidence and clinical behavior of pancreatic and adrenal lesions at a single center.

INTRODUCTION: Von Hippel-Lindau disease (VHL) is a dominantly inherited multi-system syndrome. Although pheochromocytoma is the hallmark endocrine neoplasm, pancreatic lesions occur frequently, and their management can be complex. This report describes 26 patients from a single institution with pancreatic or adrenal lesions (or both) in the background of VHL. METHODS: We reviewed records for all patients treated for adrenal (type 2 VHL) or pancreatic manifestations of VHL at our institution from 1990 to 2004. RESULTS: Forty patients with VHL were identified from 24 pedigrees; 26 (65%) had adrenal or pancreatic involvement (or both). Seven patients had isolated pheochromocytomas, 16 had isolated pancreatic lesions, and 3 had both. Of the 10 patients with pheochromocytomas, 4 had bilateral adrenal involvement. All but three pheochromocytomas were diagnosed in patients aged 40 years or younger. Of the 19 patients with pancreatic lesions, 8 had cystic lesions, 8 had neuroendocrine tumors, and 3 had both. Of 11 patients with neuroendocrine tumors, 4 had metastases (3 hepatic, 1 nodal). No patient with cystic pancreatic lesions developed carcinoma. CONCLUSIONS: VHL should be familiar to the endocrine surgeon because of the high incidence (65% in our series) of visceral endocrine lesions (pancreas 40%, adrenal gland 18%, both 7%). As seen in other hereditary endocrinopathy syndromes, pheochromocytomas are frequently bilateral; therefore,close follow-up of the contralateral gland in young patients with a unilateral lesion is critical. Cystic lesions of the pancreas may be closely monitored, whereas neuroendocrine tumors should be removed because of the risk of metastasis. Pancreatic pathology is not uncommon in VHL patients with pheochromocytoma and should be sought during the preoperative assessment.

Adolescent↗
PubMed2006

Preliminary report: functional MRI of the brain may be the ideal tool for evaluating neuropsychologic and sleep complaints of patients with primary hyperparathyroidism.

BACKGROUND: The incidence, pattern, and severity of sleep disturbance and cognitive dysfunction has not been well characterized for patients with primary hyperparathyroidism (PHPT). There is no agreement on the mechanism of the development or resolution of such symptoms, and in no previous study has cerebral activity been functionally assessed and change documented following surgical cure of patients with PHPT. METHODS: We undertook a prospective analysis to obtain pilot data on 6 patients with PHPT. Functional magnetic resonance imaging (fMRI), formal neuropsychologic (NP) tests, and health-related quality of life (HRQL) measures that included sleep assessments were performed on patients before and after parathyroidectomy. Changes in cortical activation under both conflict and neutral conditions (distracting tasks) were recorded. RESULTS: Functional MRI demonstrated postoperative changes in medial prefrontal cortex activity during cognitive processing of conflict and nonconflict tasks. Further postoperative changes were noted in the dorsolateral prefrontal cortex and parietal cortex with shifts in activations. In addition to the fMRI findings, the patients demonstrated improvements in sleep and social behavior. They tended to experience less fatigue and their processing speed on cognitive tests improved. CONCLUSIONS: These data support the feasibility and willingness of patients with PHPT to undergo fMRI assessment. Preliminary findings reflected a generalized improvement in processing efficiency postoperatively compared with a patient's preoperative state, and the HRQL measures showed improved sleep. These findings mirror those expected with sleep dysfunction. Longitudinal assessment with advanced brain imaging technology, neuropsychological (NP), and sleep evaluations is warranted to further explore cognitive, sleep, and HRQL improvement after parathyroidectomy.

Adult↗
PubMed2006

Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1.

INTRODUCTION: Pancreatic endocrine tumors (PETs) occur in at least 50% of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of disease-specific mortality. However, the timing and extent of surgery for MEN1-related PETs is controversial owing to the indolent tumor growth seen in most patients and the desire to avoid complications associated with insulin dependence. To help resolve this controversy, we retrospectively analyzed the clinical characteristics, surgical treatment, and clinical outcome of patients with MEN1-related PETs. METHODS: All patients had histologic or radiographic confirmation of a PET in the setting of MEN1. Disease progression was defined radiographically as the development of new pancreatic tumors or distant metastases. Progression-free survival (PFS) and overall survival (OS) were used as the endpoints of this analysis. RESULTS: We identified 98 patients with MEN1, 55 (56%) of whom had PETs, including 27 women and 28 men with a median age of 37 years (range 8-69 years) at the time of diagnosis. Functioning PETs were present in 35 (64%) of 55 patients, and nonfunctioning tumors were present in 20 (36%). Pancreatic surgery was performed in 38 (69%) of the 55 patients; and the first operation included enucleation (n = 4), total pancreatectomy (n = 3), Whipple procedure (n = 4), and distal pancreatectomy (n = 27). The median size of the resected tumors was 2.8 cm (range 0.6-11.0 cm). Recurrent disease developed in the residual pancreas in 7 (20%) of 35 at-risk patients a median of 7.8 years after the first operation, and distant metastases occurred in 5 (14 %) of 36 surgically treated patients without distant metastasis (2 patients had distant metastases when surgery on the primary tumor was performed) at a median of 2.7 years following surgery. At last follow-up, 16 (29%) of 55 patients with PETs had died, 12 (22%) were alive with disease, 26 (47%) were alive without evidence of disease, and 1 (2%) was lost to follow-up. The median OS was 19.5 years (range 13-26 years) and was significantly longer for patients who had functioning PETs versus those with nonfunctioning tumors (P = 0.0007), for patients who underwent surgical resection of their PETs versus those who did not (P = 0.0043), and for patients with localized versus metastatic PETs at the time of diagnosis (P < 0.0001). Multivariate analysis revealed that younger age, hormonal function, and PET resection were independently associated with longer OS. CONCLUSIONS: Our data suggest that early diagnosis and surgical excision of MEN1-related PETs improves survival. However, translating these data into a surveillance strategy for the early detection of PETs is complex owing to the potential morbidity of pancreatic resection and the risk of long-term insulin dependence.

Adolescent↗
PubMed2006

Adrenocortical carcinoma.

ACC is a rare clinical entity that carries a poor prognosis; early diagnosis and complete surgical resection are associated with the improvement in patient survival. Even with appropriated diagnosis and treatment, most patients will develop recurrence and succumb to ACC because of the underlying tumor biology, the difficulty of achieving a complete resection, and the lack of effective systemic therapies. Despite its many drawbacks, mitotane continues to be a mainstay in the treatment of high-risk patients with ACC, especially those with recurrent or metastatic disease. Recent findings suggest that mitotane, combined with conventional chemotherapeutic agents, may improve survival for such patients.

Adrenal Cortex Neoplasms↗
PubMed2006

Indications for operative intervention in patients with asymptomatic primary hyperparathyroidism: practice patterns of endocrine surgery.

BACKGROUND: Currently, many patients with primary hyperparathyroidism (PHPT) are diagnosed when they are considered to be "asymptomatic." The need for parathyroidectomy in these patients has been questioned. A consensus statement drafted after the National Institutes of Health (NIH) 2002 Workshop on Asymptomatic PHPT provided guidelines for management of such patients but has been criticized for being too conservative. The purpose of this survey was to determine the impact of these guidelines on practice patterns of endocrine surgeons. METHODS: Members of the American Association of Endocrine Surgeons (AAES) were surveyed to determine whether previously published consensus guidelines for management of asymptomatic patients with PHPT are used to base the decision of whether to offer parathyroidectomy and to ascertain what parameters are considered indicators to proceed with operative intervention. AAES members were asked about the management of patients with asymptomatic PHPT, specialty characteristics, and demographics. RESULTS: Of 257 AAES members, 96 (37%) responded to the survey. Although the majority of the respondents were aware of and followed the NIH consensus conference guidelines, the majority of surgeons (80%) would operate on a patient with PHPT who did not meet these criteria but had other nonspecific symptoms. Surgeons favored operative intervention when preoperative localization studies were positive, even if the criteria of the NIH guidelines were not fulfilled. Most of the responders who would operate on all patients with PHPT, regardless of objective parameters, were surgeons with a high-volume practice (>30 parathyroidectomies per year). The presence of multiple endocrine neoplasia (MEN) syndromes did not alter the decision to operate on asymptomatic patients. CONCLUSIONS: Endocrine surgeons do not base the decision to intervene operatively in patients with PHPT solely on objective criteria. Most high-volume, experienced endocrine surgeons believe that subjective complaints warrant operative intervention.

Adult↗
PubMed2006

Parathyroid carcinoma.

PURPOSE OF REVIEW: This article reviews current knowledge on the etiology, diagnosis and treatment of parathyroid carcinoma. RECENT FINDINGS: Due to its rarity, research on the molecular etiology and treatment of parathyroid carcinoma has been slow. Mutations of the tumor suppressor gene, HRPT2, and resultant loss of expression of its gene product have been found in the majority of parathyroid cancers studied. Recent advances in the field have identified regions on several chromosomes that demonstrate loss of heterozygosity more commonly in parathyroid carcinoma than in benign parathyroid lesions. This has provided clues to the location and identity of additional tumor suppressor genes associated with the development of this cancer. SUMMARY: Parathyroid carcinoma is an extremely rare cause of primary hyperparathyroidism, accounting for fewer than 1% of cases. The etiology of parathyroid cancer is largely unknown. Associations have been made with several inherited syndromes and with specific genetic lesions. Little is known about the most appropriate management of this disease. En bloc resection at the time of initial surgery appears to provide the best chance of cure. Anecdotal experience with adjuvant chemotherapy has shown a modest and short-lived effect. External beam radiation following surgical resection, however, may increase long-term survival compared with surgery alone. Bisphosphonates and a new class of drugs known as calcimimetics have been used effectively in some patients to control the symptoms of severe hypercalcemia in a palliative setting.

Chemotherapy, Adjuvant↗
PubMed2005

Is parathyroid carcinoma indeed a lethal disease?

BACKGROUND: Parathyroid carcinoma is a rare malignancy with a wide range of aggressiveness. There is no current staging system. Our primary aim was to review the presentation, diagnosis, surgical treatment, and outcomes of patients, with the goal of assessing the incidence of death related to parathyroid carcinoma. METHODS: The authors present a retrospective chart review on patients with parathyroid carcinoma from 1975 to 2004, identified by the tumor registry of a single tertiary-care center. Diagnoses were confirmed histologically, clinical and radiographical data were recorded, and statistical analyses were performed. RESULTS: Twenty-three cases were identified. The mean patient age was 54 years. The female:male ratio was 1.5:1. Follow-up ranged from 1 month to 23 years (median, 134 months). Mean preoperative calcium was 12.9 mg/dL. Median parathyroid hormone was 290 pg/mL. Two patients (9%) had an asymptomatic presentation, and five (22%) presented with a palpable neck mass. Only nine (39%) underwent initial comprehensive en-bloc resection. Median survival was 22 years. Five- and 10-year survival was 85.9% and 69.4%, respectively. Five- and 10-year survival with en-bloc resection was 90% and 67.5%, respectively. Local resection resulted in survival rates of 82.5% and 70.7%. Three of ten deaths were attributed to parathyroid carcinoma. In recurrent disease, computed tomography and scintigraphy had localization rates of 53% and 67%, respectively, with a concordance of 22%. CONCLUSIONS: Long-term survival is possible with parathyroid carcinoma. Death associated with parathyroid carcinoma was uncommon. A staging may be warranted despite the rarity of this disease.

Adult↗
PubMed2005

Surgical treatment of non-functioning pancreatic islet cell tumors.

Pancreatic endocrine tumors (PETs) are rare neoplasms originating from the amine precursor uptake and decarboxylation (APUD) stem cells. Although the majority of PETs are sporadic, they frequently occur in familial syndromes. PETs may cause a variety of functional syndromes or symptoms of local progression if they are non-functional. General neuroendocrine tumor markers are highly sensitive in the diagnostic assessment of a PET. Imaging studies for tumor localization and staging include computer tomography (CT) scan, magnetic resonance imaging (MRI), In(111)-octreotide scan, MIBG, and endoscopic ultrasonography (EUS). Treatment of PETs often requires a multi-modality approach; however, surgical resection remains the only curative therapy for localized (non-metastatic) disease. Treatment of metastatic disease includes biologic agents, cytotoxic chemotherapy, and liver-directed therapies.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Surgical treatment of hyperparathyroidism in patients with multiple endocrine neoplasia type 1.

HYPOTHESIS: Three-gland parathyroidectomy with trans-cervical thymectomy and cryopreservation is the preferred initial surgical approach for hyperparathyroidism (HPT) in patients with multiple endocrine neoplasia type 1. DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Thirty-seven patients with multiple endocrine neoplasia type 1 who underwent 1 or more surgical procedures for HPT from January 1, 1973 to April 30, 2004. RESULTS: At initial parathyroid surgery, 16 (43%) of 37 patients had fewer than 3 parathyroid glands resected (group 1); 16 (43%) had at least 3 but fewer than 4 glands (group 2), and 5 (14%), 4 or more glands (group 3). Follow-up of at least 6 months after initial surgery was complete for 31 (84%) of 37 patients. CONCLUSIONS: Recurrent HPT in patients with multiple endocrine neoplasia type 1 is frequent if fewer than 3 glands are removed at initial parathyroidectomy. Optimal surgical intervention must balance the risk of recurrent hypercalcemia with the morbidity of permanent hypoparathyroidism. Three-gland parathyroidectomy, transcervical thymectomy, and parathyroid cryopreservation constitute our preferred initial surgical procedure.

Adolescent↗
PubMed2005

Laparoscopic resection of adrenal cortical carcinoma: a cautionary note.

BACKGROUND: While laparoscopic removal of small, benign, functioning adrenal tumors is accepted, laparoscopic resection of adrenal tumors that may be adrenal cortical carcinoma (ACC) remains controversial. METHODS: The records of all patients with ACC evaluated at a single institution from 1991 through 2004 were reviewed retrospectively. RESULTS: Among 170 patients with ACC, 153 patients underwent open anterior adrenalectomy, 6 underwent laparoscopic adrenalectomy, 1 was treated via an open flank approach, and 10 had no operation. At a median follow-up of 28 months, 115 (86%) of 133 patients who had undergone open anterior resection of primary ACC had had a recurrence. Local recurrence and peritoneal carcinomatosis were components of initial failure in 46 (35%) and 11 patients (8%), respectively. In contrast, all 6 patients who underwent laparoscopic resection of ACC had recurrences, and peritoneal carcinomatosis was a component of initial failure in 5 (83%) of them (open vs laparoscopic resection, Fisher exact test P = .0001). CONCLUSIONS: Laparoscopic resection of ACC is associated with a high risk of peritoneal carcinomatosis. Open adrenalectomy remains the standard of care for patients presenting with an adrenal cortical tumor for which ACC is in the differential diagnosis.

Adolescent↗
PubMed2005

Carcinoid tumors of the duodenum.

BACKGROUND: Carcinoid tumors of the duodenum are rare, and their natural history has not been defined. Consequently, there is no consensus on the optimal extent of surgical treatment. METHODS: The authors reviewed the records of all patients with primary carcinoid tumors of the duodenum treated at their institution from 1969 through 2004. Patients with primary periampullary tumors and gastrinomas were excluded. RESULTS: Twenty-four patients had a pathologic diagnosis of duodenal carcinoid tumor. The majority (89%) of tumors measured less than 2 cm in diameter, and most (85%) were limited to the mucosa or submucosa. Lymph node metastases were identified in the surgical specimen in 7 (54%) of 13 patients in whom lymph nodes were examined, including 2 patients with tumors smaller than 1 cm and limited to the submucosa. At a mean follow-up of 46 months, the disease-specific survival rate was 100%, and only 2 patients have had recurrences in regional lymph nodes. No patient has had distant metastases or the carcinoid syndrome. CONCLUSIONS: Carcinoid tumors of the duodenum are indolent. The presence of regional lymph node metastases cannot be predicted reliably on the basis of tumor size or depth of invasion, and their impact on survival is uncertain.

Adult↗
PubMed2005

RET proto-oncogene: a review and update of genotype-phenotype correlations in hereditary medullary thyroid cancer and associated endocrine tumors.

Hereditary medullary thyroid carcinoma (MTC) is caused by autosomal dominant gain-of-function mutations in the RET proto-oncogene. Associations between specific RET mutations (genotype) and the aggressiveness of MTC and presence or absence of other endocrine neoplasms (phenotype) are well documented. Mutations in six exons (10, 11, 13, 14, 15, and 16) located in either cysteine-rich or tyrosine kinase domains cause one of three distinctive clinical subtypes: familial MTC, multiple endocrine neoplasia (MEN) type 2A (including variants with Hirschsprung's disease and cutaneous lichen amyloidosis), and MEN 2B. Hallmarks of MEN 2A include MTC, pheochromocytoma, and hyperparathyroidism. MEN 2B is associated with an earlier onset of MTC and pheochromocytoma, the absence of hyperparathyroidism, and the presence of striking physical stigmata (e.g., coarse facies, ganglioneuromatosis, and marfanoid habitus). Familial MTC is not associated with other endocrine neoplasms; however, the accurate distinction between familial MTC and MEN 2A may be difficult in kindreds with small size, incomplete histories, or a predominance of young individuals who may not have yet fully manifested the syndrome. Genetic testing detects greater than 95% of mutation carriers and is considered the standard of care for all first-degree relatives of patients with newly diagnosed MTC. Recommendations on the timing of prophylactic thyroidectomy and the extent of surgery are based upon a model that utilizes genotype- phenotype correlations to stratify mutations into three risk levels.

Carcinoma, Medullary↗