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Janice L Pasieka

Publications and source records attributed to Janice L Pasieka.

At least 19 recordsLinked to original sources
PubMed2007

Functioning and nonfunctioning neuroendocrine tumors of the pancreas.

PURPOSE OF REVIEW: Neuroendocrine tumors of the pancreas are a small subgroup of tumors characterized by a variety of biological behaviors. Recent changes in their classification should help better define the prognosis of this diverse group of tumors. With recent advances in diagnosis and staging, the treatment options for all neuroendocrine tumors have evolved. Presented here is a review of the current-day knowledge for neuroendocrine tumors of the pancreas. RECENT FINDINGS: A consensus by leading experts in the neuroendocrine tumors field has proposed an algorithm for the diagnosis, treatment and follow-up of these rare tumors. Surgical resection remains the first-line therapy. Alternative forms of cytoreduction such as radiofrequency ablation and embolization, have increased the ability of the surgeon to debulk these tumors, resulting in improved survival and better palliation. Contrary to adenocarcinoma of the pancreas, hormonal and biotherapy offer unique treatment strategies for these rare tumors. Very recent developments utilizing radionuclide therapy hold promise for not only palliation, but may prove to be a beneficial form of adjuvant therapy. SUMMARY: Presented here is a summary of the recent literature on the diagnosis and treatment of neuroendocrine tumors of the pancreas.

Humans↗
PubMed2006

Anaplastic thyroid cancer and primary thyroid lymphoma: a review of these rare thyroid malignancies.

BACKGROUND: To review the current literature on the treatment of anaplastic thyroid cancer (ATC) and thyroid lymphoma (TL). RESULTS: Both anaplastic carcinoma (ATC) and TL represent rare forms of thyroid cancer. ATC behaves in a highly aggressive manner, resulting in significant morbidity and mortality. Multimodality therapy consisting of both radiotherapy (RT) and chemotherapy is essential in obtaining local/regional control. Although ATC has been relatively chemo resistant, newer agents such like taxotere show promise. The role of surgery in the treatment of ATC continues to evolve, presently it should be reserved for patients who have shown an initial response to multimodality therapy and in patients in whom a complete macroscopic resection can be achieved with minimal morbidity. The successful treatment of TL currently lies in accurately diagnosing the histological subtype. Both large B-cell and mixed lymphomas are best treated with multimodality therapy consisting of CHOP combined with hyper-fractioned RT. MALT lymphomas with there more indolent course may be amenable to single modality RT or total thyroidectomy if diagnosed at an early stage IE. DISCUSSION: Although both ATC and TL are rare, it is important for surgeons to be aware of the need for multimodality therapy when treating these patients and to understand the limited role surgery plays in diagnosis and treatment.

Adult↗
PubMed2006

Adrenal venous sampling in primary hyperaldosteronism: comparison of radiographic with biochemical success and the clinical decision-making with "less than ideal" testing.

BACKGROUND: Adrenal venous sampling (AVS) is used in the workup of primary hyperaldosteronism (PA). The purpose of this study was to determine the success rate of AVS and to examine the decision-making process after "less than ideal" AVS. METHODS: A total of 60 patients underwent 62 AVS for PA. Biochemical evidence of adrenal vein cannulization was analyzed with adrenal-peripheral cortisol ratios. Pathology and clinical outcomes were reviewed in patients undergoing adrenalectomy. RESULTS: Bilateral cannulization was confirmed in only 21% (pre-adrenocorticotropic hormone [ACTH] infusion) and 44% (post-ACTH infusion) AVS. Of 39 patients who underwent adrenalectomy for presumed unilateral disease, only 16 patients had "ideal" AVS, and 18 patients had only unilateral cannulization on AVS. Despite this, 11 appeared to lateralize and 7 had imaging to support unilateral disease. Postoperatively, 15 (82%) had a significant reduction in their blood pressure, and 7 (39%) of these were cured. Surgery failed in 2 patients; both were found to have bilateral hyperplasia. Bilaterally unsuccessful cannulization (n = 5) still lateralized in 3 patients, and 2 patients had nodules on computed tomography scan. All 5 patients had significant reduction in blood pressure, and 2 were cured. CONCLUSIONS: ACTH infusion during AVS enhances the biochemical evidence of adrenal vein cannulization. Following "less than ideal" AVS, clinical decisions can still be made using anatomic and partial AVS data.

Adrenal Glands↗
PubMed2005

Clinical presentation and penetrance of pheochromocytoma/paraganglioma syndromes.

CONTEXT: The identification of mutations in genes encoding peptides of succinate dehydrogenase (SDH) in pheochromocytoma/paraganglioma syndromes has necessitated clear elucidation of genotype-phenotype associations. OBJECTIVE: Our objective was to determine genotype-phenotype associations in a cohort of patients with pheochromocytoma/paraganglioma syndromes and succinate dehydrogenase subunit B (SDHB) or subunit D (SDHD) mutations. DESIGN, SETTING, AND PARTICIPANTS: The International SDH Consortium studied 116 individuals (83 affected and 33 clinically unaffected) from 62 families with pheochromocytoma/paraganglioma syndromes and SDHB or SDHD mutations. Clinical data were collected between August 2003 and September 2004 from tertiary referral centers in Australia, France, New Zealand, Germany, United States, Canada, and Scotland. MAIN OUTCOME MEASURES: Data were collected on patients with pheochromocytomas and/or paragangliomas with respect to onset of disease, diagnosis, genetic testing, surgery, pathology, and disease progression. Clinical features were evaluated for evidence of genotype-phenotype associations, and penetrance was determined. RESULTS: SDHB mutation carriers were more likely than SDHD mutation carriers to develop extraadrenal pheochromocytomas and malignant disease, whereas SDHD mutation carriers had a greater propensity to develop head and neck paragangliomas and multiple tumors. For the index cases, there was no difference between 43 SDHB and 19 SDHD mutation carriers in the time to first diagnosis (34 vs. 28 yr, respectively; P = 0.3). However, when all mutation carriers were included (n = 112), the estimated age-related penetrance was different for SDHB vs. SDHD mutation carriers (P = 0.008). CONCLUSIONS: For clinical follow-up, features of SDHB mutation-associated disease include a later age of onset, extraadrenal (abdominal or thoracic) tumors, and a higher rate of malignancy. In contrast, SDHD mutation carriers, in addition to head and neck paragangliomas, should be observed for multifocal tumors, infrequent malignancy, and the possibility of extraadrenal pheochromocytoma.

Adolescent↗
PubMed2005

Kindred spirits of the endocrines: the training of the future endocrine surgeons.

The growth of knowledge and complexity now seen in General Surgery, has led to the sub-specialization of the discipline. Although it is considered by some to have led to the fragmentation of General Surgery and the erosion of the specialty as we know it today, others would argue that it has and will continue to lead to a stronger division and a higher standard of care. Most would argue that a higher standard of care in focus areas stimulates research and research, in turn, improves the quality of education and training. Ultimately, improved education and training leads to better patient care. Organ-specific specialization such as orthopedics and urology evolved from General Surgery and demonstrates this principle. Further sub-specialization is likely inevitable, if the discipline of General Surgery is to remain a desired specialty. Endocrine surgery has evolved into a sub-specialty of General Surgery, and over the last few decades has matured as a discipline. With this maturation comes the responsibility of defining the standard of care to be provided by surgeons involved in endocrine surgery. To achieve this goal, endocrine surgical associations and societies must set the standard of training both at the residency and postgraduate level. Where we are as a sub-specialty, where we came from, and what it will take to meet this goal are discussed.

Endocrine Surgical Procedures↗
PubMed2005

Adrenal lesions assessed in the era of laparoscopic adrenalectomy: a modern day series.

BACKGROUND: To evaluate if laparoscopic adrenalectomy (LA) has changed the indications for adrenalectomy. METHODS: Retrospective analysis of patients with adrenal lesions referred from 1992 to 2004. Patients were divided into 2 groups, those before and those after the introduction of LA. RESULTS: One hundred eighty patients were assessed. Functioning lesions were found in 120 (66%) patients. Sixty patients had nonfunctioning tumors. Before the introduction of LA, 13% had primary hyperaldosteronism (PA), 23% had pheochromocytoma (Pheo), 20% had Cushing's syndrome (CS), and 7% had adrenal cortical cancer (ACC). No difference was seen in the percentage of patients referred after LA with Pheo (20%), CS (16%), or ACC (6%). There was, however, a significant increase in the percentage of patients referred with PA after introducing LA (13% vs. 27%), P < .05. CONCLUSION: Indications for adrenalectomy have not changed since introduction of LA. There was, however, an increased percentage of patients with PA referred since the introduction of LA.

Adrenal Gland Neoplasms↗
PubMed2005

Biochemical assessment of niacin deficiency among carcinoid cancer patients.

OBJECTIVE: Carcinoid cancer patients often have elevated levels of serotonin or its precursor 5-hydroxytryptophan. Normally, serotonin synthesis accounts for a small fraction of tryptophan catabolism, which should be directed along a pathway that allows partial conversion to niacin; hence, increased diversion of tryptophan toward serotonin could cause variable degrees of niacin deficiency in carcinoid patients. Therefore, the prevalence of niacin deficiency among carcinoid patients was investigated by clinical assessment of pellagra and biochemical assessment of whole blood niacin number, a ratio derived from two biologically active forms of niacin (NAD/NADP x 100). METHODS: Clinical and biochemical niacin status were assessed in a cohort of newly diagnosed carcinoid patients with carcinoid syndrome (CCS, n = 36), carcinoid patients without carcinoid syndrome (CWCS, n = 32) and noncarcinoid controls (n = 24) recruited at two primary care clinics. Other aspects of serotonin metabolism were measured by analyses of plasma serotonin and tryptophan and urinary excretion of 5-hydroxyindoleacetic acid. RESULTS: Biochemical niacin deficiency (niacin number < 130) was significantly more common in CCS patients (10 out of 36) compared to controls (p < 0.05, Fisher's exact test), while CWCS patients displayed an incidence that was not significantly elevated (4 out of 32). Only one CCS patient, who was also identified biochemically as niacin deficient, was clinically diagnosed with pellagra. CONCLUSION: Biochemical niacin deficiency is more prevalent among newly diagnosed CCS patients than in controls. Manifestation of pellagra is a less sensitive indicator, and dependence on this endpoint could lead to a lack of appropriate nutritional support for this group of patients.

Adult↗
PubMed2004

A review of thyroid cancer with intermediate differentiation.

BACKGROUND AND OBJECTIVES: Tall cell (TCV), columnar cell (CCV), insular (IC), diffuse sclerosing (DSPTC) and solid/trabecular are uncommon subtypes of thyroid cancer, which have generally been described in case reports or small series in the world literature. Due to the rarity of these thyroid cancers, their clinical behavior remains incompletely understood. The aim of this review was to pool the currently available clinical information regarding these uncommon thyroid cancers so as to gain a better understanding of their clinical aspects and natural history. METHODS: A computer-aided search of MEDLINE (1966-2001, PUBMED website) and CINAHL (1982-2001) databases was performed, as well as a review of the reference section of each primary study was done. All cases of TCV, CCV, DSPTC, solid/trabecular, and IC described in the English medical literature were identified. For the subtypes DSPTC, TCV, and IC, clinical data from the published case series were combined in a weighted analysis. Weighting was based on the number of cases per series. For the CCV and the solid/trabecular variant, due to the small number of cases, raw figures for the clinical features were obtained. RESULTS: DSPTC (n = 65) appeared to have a tendency for intra-thyroidal extension (40%) and a high propensity for nodal metastates (68%). The mean overall tumor related mortality was similar to well differentiated thyroid cancer (WDTC) at only 2% at 8 years follow-up. The solid/trabecular variant was seen in 37% of the radiation induced thyroid tumors of the Chernobyl accident. It had a high propensity for extrathyroidal extension, and cervical lymph node metastases were found in up to 83% of patients. Unlike WDTC, TCV (n = 209) was a more aggressive tumor, associated with distant metastases in 22% of cases and had a mean tumor related mortality of 16%. The histological diagnosis of TCV was a poor prognostic factor regardless of patient age or tumor size. The CCV (n = 41) had a high overall mortality rate of 32%. When encapsulated, however, CCV had an excellent prognosis similar to that found in WDTC. In contrast, CCV tumors that were not encapsulated had extrathyroidal spread in 67% and had distant metastases in 87% of patients. The variant of IC (n = 213) appeared to be an aggressive subtype of thyroid cancer. The mean loco-regional recurrence and/or distant metastases rate was 64% and tumor related mortality was high at 32%. CONCLUSIONS: DSPTC, TCV, CCV, and IC are thyroid cancer subtypes, which have a biological aggressiveness, which appear to be intermediate between that of WDTC and poorly differentiated anaplastic thyroid cancer. J. Surg. Oncol. 2004;86:44-54.

Humans↗
PubMed2004

131I-meta-iodobenzylguanidine in the management of metastatic midgut carcinoid tumors.

The management of metastatic neuroendocrine tumors incorporates multimodal therapy with surgery, biotherapy, and chemotherapy. Tumor-targeted therapies using radiolabeled octreotide and metaiodobenzylguanidine (mIBG) represent a novel treatment approach. The aim of this study was to evaluate the effectiveness of 131I-mIBG in the treatment of metastatic midgut carcinoid tumors. survival outcomes were assessed for patients treated at two regional cancer centers and then compared. One center used 131I-mIBG routinely in the management of metastatic carcinoid tumors (center A), and the other did not use this modality (center B). Only patients with histologically proven metastatic carcinoid tumor shown, or thought most likely, to be of midgut origin were included in the study. During the period 1980 to 2002, a series of 58 patients from center A with metastatic carcinoid tumor arising from the midgut underwent multimodality therapy with the addition of 131I-mIBG. Their median age was 64 years. The median dose of 131I-mIBG administered was 6751 MBq, and there was an average of 2.8 treatments per patient. During the same period, 58 patients with metastatic carcinoid were treated at center B with similar multimodality therapy without the use of 131I-mIBG therapy. Their median age was 65 years. Survivals at 3 and 5 years were 77% and 63%, respectively (95% CI 47-75), for group A. The 3- and 5-year survivals for group B were 56% and 47% (95% CI 34-59), respectively. The mean follow-up was 6.6 years for group A and 5.0 years for group B. Although retrospective in nature, this study suggests that the addition of 131I-mIBG therapy to the treatment protocol of patients with metastatic midgut carcinoid tumors prolongs survival.

3-Iodobenzylguanidine↗
PubMed2004

Primary thyroid lymphomas.

Primary thyroid lymphoma is a rare disease that continues to produce diagnostic and therapeutic dilemmas. There was great difficulty in distinguishing thyroid lymphoma from anaplastic thyroid carcinoma but, because of new immunocytochemical staining techniques and increased cytopathologic knowledge, our ability to diagnose thyroid lymphoma has improved drastically over the past decade. Surgery that was once the mainstay of treatment for this disease, now plays a minimal role. Current treatment regimens for primary thyroid lymphoma consist of chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone) and external beam radiation. The overall and distant relapse rates have been shown to be significantly lower in those patients receiving combined modality therapy compared to chemotherapy or radiation alone. Although the role of surgery has changed over time, it continues to play an important role, especially in confirming diagnoses through open biopsies, potentially providing local control in the more indolent subtypes, and may play a role in the palliation of symptoms for large obstructive lymphomas. The evolving classification of extranodal lymphomas has brought about a better understanding of the biologic behavior of these tumors. Most thyroid lymphomas are B-cell origin, with six different histologic subtypes, but there appears to be two distinct clinical and prognostic groupings of these rare tumors. The more indolent lymphomas are the subgroup of mucosa-associated lymphoid tissue (MALT) lymphomas comprising approximately 6% to 27% of thyroid lymphomas. This subgroup, when localized to the thyroid (stage IE), responds well to total thyroidectomy or radiation with a complete response rate of more than 90%, leading some authors to recommend surgery as primary therapy in the treatment of localized MALT lymphomas. Therefore, surgery as a primary treatment for thyroid lymphomas would only be recommended under ideal conditions, such as MALT subtype stage IE only, and completely resectable with minimal morbidity. Unfortunately, this scenario is rarely the case. The more common subtype, comprising up to 70% of cases, is diffuse large B-cell lymphoma. This subtype appears to have the most aggressive clinical course with almost 60% of these tumors diagnosed with disseminated disease. Up to 40% of all diffuse large cell lymphomas appear to have undergone transformation from a MALT lymphoma, but they behave in a similar fashion to diffuse large cell lymphomas. Treatment for these tumors should include chemotherapy and radiation. The overall 5-year survival for this aggressive group is less then 50%. Surgery is rarely beneficial in diffuse large cell lymphoma and the mixed large cell subtypes because the disease is generally disseminated and surgical excision of all disease is not possible or associated with increased morbidity. However, there may be a role for palliative surgical debulking to alleviate obstructive symptoms while the patient is undergoing standard chemotherapy and radiation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Asymptomatic primary hyperparathyroidism: a surgical perspective.

Since the advent of multichannel autoanalyzers and routine screening of serum calcium levels, prevalence of primary hyperparathyroidism (pHPT) has increased to between 0.1% and 0.4%. As more patients present with "asymptomatic" pHPT, ideal treatment of "mild" disease becomes more controversial, with the possibility of safe, nonoperative management in a selected group of patients. Accumulated evidence confirms that the majority of these patients suffer from vague, nonspecific complaints that are very real and can improve following parathyroidectomy. Furthermore, parathyroidectomy in patients with pHPT has been demonstrated to improve bone mineral density, reduce fracture risk, and improve health-related quality of life and possibly overall survival. Therefore, all patients with primary hyperparathyroidism should be referred for surgical evaluation by an experienced endocrine surgeon.

Bone Diseases↗
PubMed2004

The palliative role of 131I-MIBG and 111In-octreotide therapy in patients with metastatic progressive neuroendocrine neoplasms.

BACKGROUND: Radiolabeled octreotide and metaiodobenzylguanidine (MIBG) have demonstrated limited antitumor effect on neuroendocrine neoplasms (NENs). The purpose of this study was to assess the palliative benefit of radionuclide therapy (RNT) in NENs. METHODS: Since April 2001, RNT for progressive, nonsurgically resectable NENs was utilized. NENs that were MIBG positive received 131 I-MIBG therapy, whereas octreotide-only-positive neoplasms received 111 In-octreotide therapy. Symptomatic, quality of life (QoL), biochemical, and radiographic responses to RNT were evaluated. RESULTS: Twenty-four patients had either MIBG or octreotide avid NENs. A mean (range) of 4 (1-7) 131 I-MIBG treatments were given to 13 patients over a duration of 18 months (6-42). The group consisted of 2 medullary thyroid cancer (MTC), and 2 foregut and 9 small-bowel carcinoids; 12 patients (92%) had symptomatic improvement. Stability of tumor size was confirmed in 6, regression in 2, and progression in 5. Biochemical responses were demonstrated in 2. Five (2-16) treatments of 111 In-octreotide was given to 11 patients over 17 months (6-40). There were 1 MTC, 1 insulinoma, 2 gastrinomas, and 3 small-bowel and 4 foregut carcinoids. Symptomatic benefit was seen in 6 (55%), biochemical response in 3. Tumor regression was seen in 1, stability in 5, and progression in 5. CONCLUSIONS: Radionuclide therapy appears to offer good palliation to patients with progressive NENs.

3-Iodobenzylguanidine↗
PubMed2003

Functional results and quality of life after tracheal resection for locally invasive thyroid cancer.

BACKGROUND: Local invasion of the upper aerodigestive tract by thyroid cancer, although uncommon, is a serious cause of morbidity and mortality. The impact of aerodigestive tract resection on the functional status and quality of life of the patient has not previously been investigated. METHODS: Patients with locally invasive thyroid cancer were included in a prospective surgical protocol. Swallowing function was assessed with barium swallow at 7 days and 1 month postoperatively. Postoperative quality of life (QOL) was measured using a validated head and neck QOL instrument. RESULTS: Seven patients underwent airway resection for locally invasive recurrent thyroid cancer in the period 1999 to 2001. At 1 week postoperative 3 of 7 (43%) had no evidence of aspiration on barium swallow. At 4 weeks 6 of 7 (86%) had no aspiration. Postoperative QOL scores in the domains of eating function (85.2) and emotional status (78.6) were significantly better than those of a comparison group undergoing treatment for cancers of the oropharynx, P = 0.012 and P = 0.0077, respectively. CONCLUSIONS: Tracheal resection for locally invasive thyroid cancer is associated with a return to full dietary intake within 4 weeks of surgery in most cases. Function and QOL after this type of surgery are acceptable.

Aged↗
PubMed2003

Anaplastic thyroid cancer.

Anaplastic thyroid carcinoma is one of the most aggressive malignancies, with a poor prognosis. Although rare, representing only 2% of clinically recognized thyroid cancers, the overall median survival is limited to months. Most patients are elderly and seek treatment with a rapidly growing mass. Almost half the patients seek treatment with distant metastases, with as many as 75% developing distant disease during their illness. In most the patients, complete surgical resection is not possible. There are, however, a few patients with resectable disease reported in the literature who have demonstrated long-term survival with aggressive multimodal therapy that included surgery, radiation, and chemotherapy. Preclinical studies in human anaplastic thyroid carcinoma cell lines show promise that new approaches to the management of this disease will be found in the future. Until such time when an effective regimen is found, all patients with anaplastic thyroid carcinoma should be evaluated for multimodal therapy in the setting of a clinical trial.

Carcinoma↗
PubMed2002

Patient-based surgical outcome tool demonstrating alleviation of symptoms following parathyroidectomy in patients with primary hyperparathyroidism.

This study assessed the impact of parathyroidectomy on the preoperative symptoms of patients with primary hyperparathyroidism (1 degrees HPT) using a surgical outcome tool designed specifically for HPT. The multicenter nature of this study allowed us to validate further this disease-specific outcome tool. 1 degrees HPT patients from Canada, the United States, and Australia filled out the questionnaire preoperatively and postoperatively on day 7 and at 3 and 12 months. The symptoms recorded by the patients were expressed as parathyroidectomy assessment of symptoms (PAS) scores: the higher the score, the more symptomatic is the patient. Quality of Life (QOL) and self-rated health uni-scales were included. Altogether, 203 patients with 1 degrees HPT were enrolled; 27 from center A, 54 from center B, and 122 from center C; 58 nontoxic thyroid patients were enrolled for comparison. The comparison group had no significant change in their PAS scores throughout the study (scores 184, 215, 156, 186). All three centers demonstrated a significant reduction in symptoms following surgery. The median preoperative PAS score from center B patients was 282. Following surgery, PAS scores decreased significantly: 136, 58, 0 (p <0.05). Center C patients had a median preoperative PAS score of 344, decreasing postoperatively to 228 (p <0.05) and continuing to decrease to 190, then 180. Center A also demonstrated a significant reduction in symptoms at 3 months, from 510 preoperatively to 209 (p <0.001). Both QOL and self-rated health improved in the HPT patients, whereas no change was found in the comparison group following surgery. PAS scores are a reliable, disease-specific measure of symptoms seen with HPT. Parathyroidectomy significantly reduces these preoperative symptoms, and this change translated into an improved health-related QOL for the patients.

Adolescent↗