Adrenal ganglioneuroma: features and outcomes of 27 cases at a referral cancer centre.

BACKGROUND: Adrenal ganglioneuroma (AGN) is a rare neurogenic tumour that can mimic other adrenal neoplasms. Limited information, mostly derived from small cases series, is available for AGN. METHODS: A retrospective review for AGNs seen at a tertiary referral centre describing important features to distinguish AGN from other adrenal neoplasms. RESULTS: Of 53 ganglioneuromas, 27 were AGNs. Median age was 31 years (range, 1·7-64 years) and median tumour size was 8 cm (range, 1·5-20 cm). Seventeen AGNs (63%) were detected incidentally and nine patients (33%) presented with abdominal/back discomfort. Catecholamine levels, available for 21 patients, were normal. On computed tomography (CT), most AGNs were homogenous and well circumscribed with a median density of 32·5 Hounsfield units (HU) on unenhanced CT; 40 HU on postcontrast venous phase; and 66·5 HU on delayed postcontrast phase. On magnetic resonance imaging (MRI), AGNs had hypo-intense signal on T1-weighted images with heterogeneous hyperintense signal on T2-weighted images. In four patients, there was no tumour growth during median follow-up of 48 months (range, 21-60 months). One patient had malignant peripheral nerve sheath tumour arising from AGN. Thirteen patients with resected AGN had no recurrence during a median follow-up of 50 months (range, 2-135 months). CONCLUSIONS: We herein describe the largest AGN series reported to date. Isolated AGNs do not produce catecholamines and have CT imaging characteristics that can help in distinguishing them from other adrenal and para-adrenal neoplasms. The natural history of AGNs is usually benign, although local extra-adrenal extension or malignant transformation can rarely occur.

Tibolone increases bone mineral density but also relapse in breast cancer survivors: LIBERATE trial bone substudy.

INTRODUCTION: The Livial Intervention Following Breast Cancer: Efficacy, Recurrence and Tolerability Endpoints (LIBERATE: Clinical http://Trials.gov number NCT00408863), a randomized, placebo-controlled, double-blind trial that demonstrated that tibolone (Livial), a tissue-selective hormone-replacement therapy (HRT), increased breast cancer (BC) recurrence HR 1.40 (95% CI, 1.14 to 1.70; P = 0.001). A subgroup of women was entered into a study of bone mineral density (BMD). METHODS: Women with surgically excised primary BC (T1-3, N0-2, M-0) within the last 5 years, complaining of vasomotor symptoms, were assigned to tibolone, 2.5 mg daily, or placebo treatment for a maximum of 5 years. The BMD substudy enrolled 763 patients, using dual-energy X-ray absorptiometry (DXA) scanning at baseline and at 2 years. RESULTS: In the bone substudy, 699 of 763 women were eligible (345 allocated to tibolone, and 354, to placebo). After undergoing DXA scans, 300 (43%) women had normal BMD; 317 (45%), osteopenia; and 82 (11.7%), osteoporosis. Low body-mass index (P < 0.001), Asian race (P < 0.001), and late age at menarche (P < 0.04) predicted low bone mass at baseline. Tibolone increased BMD by 3.2% at the lumbar spine and 2.9% at the hip compared with placebo (both P < 0.001). The majority of fractures (55%) occurred in osteopenic patients. Women with normal BMD had increased recurrence with tibolone, 22 (15.6%) of 141 compared with placebo, 11 (6.9%) of 159 (P = 0.016), whereas no increased BC recurrence was seen in women with low BMD; 15 (7.4%) of 204 taking tibolone versus 13 (6.7%) of 195 taking placebo. CONCLUSIONS: Tibolone is contraindicated after BC treatment, as it increases BMD and BC recurrence. Risk of BC recurrence was elevated in BC women with normal BMD (compared with low) who took tibolone.

Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: the University of Texas MD Anderson Cancer Center Experience.

BACKGROUND: Cushing syndrome (CS) secondary to ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) has been described in association with a variety of tumors. The current experience with this syndrome was based on a few case series and individual case reports. Limited data were available about the tumors associated with CS-EAS in a cancer center setting. In this report, the authors have described their experience with CS-EAS at The University of Texas MD Anderson Cancer Center to further enhance the current understanding and management of this syndrome. METHODS: This was a retrospective review of 43 patients with CS-EAS who were diagnosed between 1979 and 2009 at The University of Texas MD Anderson Cancer Center. RESULTS: Different neuroendocrine tumors were associated with CS-EAS. Twenty-one patients (48.9%) had tumors located in the chest cavity, with bronchial carcinoid and small cell lung cancer representing the 2 most common causes. The ACTH source remained occult in 4 patients (9.3%) despite extensive workup. Clinical presentation varied, and the classic features of CS were not evident in some patients. Death occurred in 27 patients (62.8%), and the median overall survival was 32.2 months. Major morbidities included new-onset or worsening hyperglycemia (77%), symptomatic venous thromboembolism (14%), and infections (23%). CONCLUSIONS: In patients with CS-EAS who attended a comprehensive cancer center, tumors originating in the chest cavity were the leading tumors associated with this syndrome. The authors suspect that CS-EAS is under reported because of the atypical presentation in some patients. Thus, they suggest careful evaluation of patients with neuroendocrine tumors to avoid missing coexisting CS-EAS.

Distant Metastases From Childhood Differentiated Thyroid Carcinoma: Clinical Course and Mutational Landscape.

CONTEXT: Distant metastases (DM) from childhood differentiated thyroid carcinoma (DTC) are uncommon and published studies are limited. OBJECTIVE: This work aimed to describe the outcomes of patients with DM from childhood DTC and to evaluate the molecular landscape of these tumors. METHODS: A retrospective study was conducted at a tertiary cancer center including patients with pediatric DTC (diagnosed at age ≤ 18 years from 1946 to 2019) and DM. RESULTS: We identified 148 patients; 144 (97%) had papillary thyroid carcinoma (PTC) and 104 (70%) were female. Median age at DTC diagnosis was 13.4 years (interquartile range [IQR], 9.9-15.9 years). Evaluable individuals received a median of 2 (IQR, 1-3) radioactive iodine (RAI) treatments at a median cumulative administered activity of 238.0 mCi (IQR, 147.5-351.0 mCi). The oncogenic driver was determined in 64 of 69 PTC samples: RET fusion (38/64; 59%), NTRK1/3 fusions (18/64; 28%), and the BRAF V600E mutation (8/64; 13%). At last evaluation, 93% had persistent disease. The median overall and disease-specific survival after DTC diagnosis were 50.7 and 52.8 years, respectively. Eight (5%) PTC patients died of disease after a median of 30.7 years (IQR, 20.6-37.6 years). CONCLUSION: Childhood DTC with DM persists in most patients despite multiple courses of RAI, but disease-specific death is uncommon, typically occurring decades after diagnosis. Fusion genes are highly prevalent in PTC, and all identified molecular alterations have appropriate targeted therapies. Future studies should focus on expanding genotype-phenotype correlations, determining how to integrate molecularly targeted therapy into treatment paradigms, and relying less on repeated courses of RAI to achieve cure in patients with DM from childhood DTC.

Safety and efficacy of tibolone in breast-cancer patients with vasomotor symptoms: a double-blind, randomised, non-inferiority trial.

BACKGROUND: Vasomotor symptoms and bone loss are complications frequently induced by adjuvant treatment for breast cancer. Tibolone prevents both side-effects, but its effect on cancer recurrence is unknown. The aim of this study was to show non-inferiority of tibolone to placebo regarding risk of recurrence in breast-cancer patients with climacteric complaints. METHODS: Between July 11, 2002, and Dec 20, 2004, women surgically treated for a histologically confirmed breast cancer (T(1-3)N(0-2)M(0)) with vasomotor symptoms were randomly assigned to either tibolone 2.5 mg daily or placebo at 245 centres in 31 countries. Randomisation was done by use of a centralised interactive voice response system, stratified by centre, with a block size of four. The primary endpoint was breast-cancer recurrence, including contralateral breast cancer, and was analysed in the intention-to-treat (ITT) and per-protocol populations; the margin for non-inferiority was set as a hazard ratio of 1.278. This study is registered with ClinicalTrials.gov, number NCT00408863. FINDINGS: Of the 3148 women randomised, 3098 were included in the ITT analysis (1556 in the tibolone group and 1542 in the placebo group). Mean age at randomisation was 52.7 years (SD 7.3) and mean time since surgery was 2.1 years (SD 1.3). 1792 of 3098 (58%) women were node positive and 2185 of 3098 (71%) were oestrogen-receptor positive. At study entry, 2068 of 3098 (67%) women used tamoxifen and 202 of 3098 (6.5%) women used aromatase inhibitors. The mean daily number of hot flushes was 6.4 (SD 5.1). After a median follow-up of 3.1 years (range 0.01-4.99), 237 of 1556 (15.2%) women on tibolone had a cancer recurrence, compared with 165 of 1542 (10.7%) on placebo (HR 1.40 [95% CI 1.14-1.70]; p=0.001). Results in the per-protocol population were similar (209 of 1254 [16.7%] women in the tibolone group had a recurrence vs 138 of 1213 [11.4%] women in the placebo group; HR 1.44 [95% CI 1.16-1.79]; p=0.0009). Tibolone was not different from placebo with regard to other safety outcomes, such as mortality (72 patients vs 63 patients, respectively), cardiovascular events (14 vs 10, respectively), or gynaecological cancers (10 vs 10, respectively). Vasomotor symptoms and bone-mineral density improved significantly with tibolone, compared with placebo. INTERPRETATION: Tibolone increases the risk of recurrence in breast cancer patients, while relieving vasomotor symptoms and preventing bone loss. FUNDING: Schering-Plough (formerly NV Organon, Oss, Netherlands).

Hypercortisolemia and infection.

Hypercortisolemia is a condition involving a prolonged excess of serum levels of cortisol that can develop as a result of disregulatory abnormalities in the hypothalamic-pituitary-adrenal axis or from exogenous-source steroids. Hypercortisolemia induces a state of immunocompromise that predisposes the patient to various bacterial, viral, fungal, and parasitic infections. To ensure optimal management of hypercortisolemia, the primary clinician must be cognizant of its different causes and aware of the different infections associated with cortisol excess. In the hypercortisolemic patient, it is necessary to restore normal cortisol levels to reduce the risk of infection or to improve the control and cure of established infection.

Health profiles of 814 very long-term breast cancer survivors.

BACKGROUND: Information regarding the long-term health impact of cancer and cancer treatments on survivors is gradually accumulating but is generally limited to the first few years after diagnosis. PATIENTS AND METHODS: We analyzed health information provided by 814 breast cancer survivors whose cancer was diagnosed >or= 15 years earlier and compared the information with that of female survivors of other cancers. These women were identified from a larger cohort of very long-term survivors of cancer who responded to a health survey. RESULTS: All survivors underwent surgery as part of their cancer therapy, and 334 (41%) also received chemotherapy. Survivors of breast cancer reported significantly more arthritis/osteoporosis, cataracts, and heart problems than other cancer survivors. Those who were treated with chemotherapy reported more psychologic problems, loss of memory, and circulation problems than those who did not receive chemotherapy. A third (30.4%) of the breast cancer survivors reported that cancer had affected their health. CONCLUSION: Very long-term survivors of breast cancer generally report good health and quality of life, as do very long-term survivors of other cancers. Nevertheless, 30.4% of breast cancer survivors report that cancer has affected their health. A number of distinctions are noted that can serve a basis for further hypothesis-driven research.

Health profiles of 996 melanoma survivors: the M. D. Anderson experience.

BACKGROUND: The incidence and survival of melanoma are increasing, but little is known about its long-term health effects in adult survivors. METHODS: A health survey was available from 996 melanoma survivors (577 treated with surgery alone, and 391 with combined treatments). Their medical/physiologic and psychosocial responses were analyzed and compared with those of the survivors from other cancers. RESULTS: The melanoma survivors were 44.8 +/- 12.8 years of age at diagnosis (significantly younger than the survivors of other cancers) and 63.7 +/- 12.8 years at survey. Melanoma survivors were less likely to report that cancer had affected their health than survivors of other cancers (15.8% vs. 34.9%). The 577 individuals treated with surgery alone reported arthritis/osteoporosis, cataracts, and heart problems most frequently (less often than survivors of other cancers). The 391 individuals who had undergone combined treatments reported circulation problems and kidney problems generally as often as survivors of other cancers. Health problems were not associated with number of decades since diagnosis but with age at diagnosis, treatment modality, and family relationships. CONCLUSION: We present information from a large cohort of long-term survivors of melanoma. As a group, they were less likely to report that cancer had affected their overall health than survivors of other cancers; a number of disease related and psychosocial factors appear to influence their health profiles.

Contribution of routine chest x-ray in the long-term follow-up of patients with differentiated thyroid carcinoma.

CONTEXT: Chest x-ray (CXR) is performed routinely in patients with differentiated thyroid cancer (DTC) but its importance is not well defined. OBJECTIVE: Determine the contribution of routine CXR in the detection of intrathoracic metastases in patients with DTC on follow-up. DESIGN: Retrospective chart review. SETTING: Comprehensive cancer center. PATIENTS: PATIENTS with localized DTC, seen at the University of Texas M. D. Anderson Cancer Center over a 20-year period. MAIN OUTCOMES: CXR abnormality developed in 6.6% of patients. RESULTS: Of 333 patients, (298 papillary and 35 follicular thyroid carcinoma), 22 patients (6.6%) (18 papillary and 4 follicular) developed abnormal CXR. Median interval to CXR conversion was 66 months (range, 8-228 months). Most had additional evidence of disease at the time of CXR conversion. At last follow-up, 9 were alive with disease, 7 had died of other etiology, and 6 had died of DTC. Of 311 patients with normal CXRs throughout, (280 papillary and 31 follicular) 226 had no evidence of disease, 71 were alive with disease, and 14 had died of other etiology at last follow-up. CONCLUSIONS: In the absence of other evidence of disease, the contribution of CXR is limited in the long-term follow-up of patients with DTC.

Preexisting adrenal masses in patients with adrenocortical carcinoma: clinical and radiological factors contributing to delayed diagnosis.

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy that is usually large (>5 cm) at time of diagnosis. Delayed diagnosis significantly worsens survival. We describe adrenal gland morphology prior to ACC diagnosis and discern potential causes of delayed diagnosis. ACC patients seen at The University of Texas MD Anderson Cancer Center between 1998 and 2014 who had cross-sectional body imaging ≥3 months prior to their diagnosis. We conducted a detailed review of clinical and radiological features in these patients prior to ACC diagnosis. Of 439 patients with ACC, 25 had imaging preceding ACC diagnosis (5 with normal adrenal glands and 20 with preexisting masses). On the first available images, the median mass size was 2.8 cm (range 0-9) with median precontrast density of 36 Hounsfield units (range 17-43) and became 9 cm (range 1-18) at the time of ACC diagnosis. The median interval between first available image and ACC diagnosis was 20 months (range 3-89). In the 5 patients whose initial images showed normal adrenal glands, the time between the last normal scan and ACC diagnosis ranged from 5 to 36 months. The most common reason for delayed ACC diagnosis was the presumed benign status of the preexisting mass (n = 13, 65 %). Radiologically suspicious adrenal masses can precede ACC diagnosis and have variable growth patterns. ACC can also develop de novo within a few months in a radiologically documented normal adrenal gland. The presumed benignancy of preexisting masses based on size is the main reason for delayed ACC diagnosis.

Primary hyperparathyroidism and osteosarcoma: examination of a large cohort identifies three cases of fibroblastic osteosarcoma.

UNLABELLED: To study a possible relationship between hyperparathyroidism and osteosarcoma, we reviewed 1234 osteosarcoma patients. In this cohort, only three patients had a diagnosis of both hyperparathyroidism and fibroblastic osteosarcoma. These results indicate that hyperparathyroidism is not more prevalent in patients with osteosarcoma than in the general population. However, the presence of hyperparathyroidism may modify the histologic and cytologic features of osteosarcoma. INTRODUCTION: The finding of osteosarcoma in rats receiving human PTH(1-34) raised the question of whether hyperparathyroidism might be a risk factor for development of osteosarcoma in humans. MATERIALS AND METHODS: To study a possible relationship between hyperparathyroidism and osteosarcoma, we reviewed the medical records of 1234 osteosarcoma patients seen at The M.D. Anderson Cancer Center since 1948. Our study focused on clinical, biochemical, radiologic, and histopathologic findings indicative of primary hyperparathyroidism and the features of osteosarcoma. RESULTS: Of the 1234 cases reviewed, 3 patients had a diagnosis of both primary hyperparathyroidism and osteosarcoma. In two cases, hyperparathyroidism preceded the osteosarcoma, and in one case, both conditions were diagnosed at the same time. In two cases with concomitant hyperparathyroidism and osteosarcoma, features of osteitis fibrocystica were identified. The third patient was treated for hyperparathyroidism 3 years before osteosarcoma was diagnosed. All three patients had histologic features of fibroblastic osteosarcoma, a type that accounts for no more than 20% of osteosarcomas. To assess whether the prevalence of hyperparathyroidism was greater than expected in the normal population, we compared the age- and sex-specific prevalence in our cohort to a population of healthy individuals in Tromso, Norway. This analysis showed no significant differences between the two populations, despite the fact that a higher prevalence of hyperparathyroidism (6.9% versus 1.6%) was noted in the 60- to 69-year-old female osteosarcoma age group. CONCLUSIONS: Our results indicate that hyperparathyroidism is not more prevalent in affected individuals with osteosarcoma than in the general population. The finding of fibroblastic osteosarcoma in all three patients raises the question of whether coexistent hyperparathyroidism may modify the cytologic and histologic features of the malignancy.

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.

Hearing loss among cancer survivors.

Cancer therapies may cause hearing loss (HL) in some patients. The purpose of this study is to examine at risk factors for HL and its impact on the health of a large cohort of cancer survivors. This is a descriptive, cross-sectional study of long-term cancer survivors who reported that they have experienced HL as a result of their cancer. Of 3571 respondents who answered a mailed survey, 243 (6.8%) reported HL. We analyzed the responses to discern the potential impact of demographics, cancer type or disease treatments on hearing, as well as the potential impact of HL on socioeconomic parameters (education, family and work). Survivors of head and neck cancer, sarcoma and testicular cancer reported HL most frequently. Among the younger survivors, the frequency of HL was higher than age-matched persons from the general U.S. population. Cancer survivors with HL were more likely to report that cancer had affected their overall health (71 vs. 32%) and were unable to work. While cisplatinum exposure was noted more frequently in respondents with HL, no other treatments, including radiotherapy, were shown to have a significant impact on hearing. There were no differences with respect to age, gender or family dynamics. Hearing loss affects a minority of long-term cancer survivors and may have an impact on their education, ability to work and overall health.

Hormone replacement therapy and lung cancer risk: a case-control analysis.

PURPOSE: To date, there are few published data regarding the use of hormone replacement therapy (HRT) and lung cancer risk. Therefore, we analyzed data regarding HRT use from a large case-control study designed to study genetic susceptibility to lung cancer to determine whether HRT affected risk of lung cancer. EXPERIMENTAL DESIGN: In a secondary analysis, we compared self-reported HRT use among 499 women with lung cancer and 519 healthy age-matched controls. RESULTS: HRT use was associated with an overall reduced risk of 34% [odds ratio (OR), 0.66; 95% confidence interval (CI), 0.51-0.89] of lung cancer, after adjusting for age, ethnicity, smoking status, education, body mass index, and menopausal status. The use of estrogen replacement therapy alone was associated with a 35% reduction in lung cancer risk (OR, 0.65; 95% CI, 0.47-0.89) and the use of combination therapy (estrogen and progestin) was associated with a 39% reduction in lung cancer risk (OR, 0.61; 95% CI, 0.40-0.92). HRT use was also associated with a statistically significantly reduced risk of lung cancer in current smokers (OR, 0.59; 95% CI, 0.38-0.92), but the risk estimates were not statistically significant in never (OR, 0.72; 95% CI, 0.37-1.40) or former smokers (OR, 0.73; 95% CI, 0.46-1.15). In addition, as the cigarette pack-years increased among ever smokers, the protective effect diminished, so that light smokers appeared to benefit the most from HRT use. Decreased lung cancer risks were also evident when the data were stratified by age, ethnicity, and body mass index. The joint effects of HRT use and mutagen sensitivity suggest that HRT use modifies lung cancer risk for genetically susceptible women. HRT use was also associated with a lower risk of death and improved survival compared with the women not taking HRT. To provide a possible biological mechanism to explain our findings, we compared plasma levels of insulin-like growth factor I in users and nonusers, and demonstrated that HRT use was associated with statistically significantly lower insulin-like growth factor I levels for both cases and controls compared with non-HRT users. CONCLUSIONS: These data suggest an association of HRT use with a decrease in lung cancer risk. However, there are several limitations to this secondary analysis, requiring that the data be viewed with caution, and confirmation is required in well-designed hypothesis driven studies. The biological role of HRT in lung cancer remains understudied, and only extensive research can yield new insights into the mechanisms underlying a protective effect of HRT for lung cancer.

A novel point mutation of the RET protooncogene involving the second intracellular tyrosine kinase domain in a family with medullary thyroid carcinoma.

Hereditary medullary thyroid carcinoma, a tumor that arises from the parafollicular cells of the thyroid gland, occurs in isolation (as in familial medullary thyroid carcinoma), in association with hyperparathyroidism and pheochromocytoma (as in multiple endocrine neoplasia type 2A), or in association with pheochromocytoma, marfanoid habitus, and mucosal neuromas (as in multiple endocrine neoplasia type 2B). These genetic syndromes are associated with germline-activating mutations of the RET protooncogene, a cell surface tyrosine kinase receptor, which is believed to modulate specific intracellular signaling pathways involved in the regulation of C cell proliferation and apoptosis. RET-activating mutations involve two important functional areas of the receptor: the cysteine-rich extracellular domain and the intracellular tyrosine kinase domain. Multiple endocrine neoplasia type 2A and familial medullary thyroid carcinoma are more commonly associated with mutations in the cysteine-rich extracellular domain, whereas multiple endocrine neoplasia type 2B is exclusively associated with mutations involving the second intracellular tyrosine kinase domain. Here, we describe a novel missense mutation of the RET protooncogene that substitutes arginine for proline at codon 912 of the intracellular tyrosine kinase domain in a family with medullary thyroid carcinoma.

Breast cancer and hormonal replacement therapy.

Understanding the association between hormone replacement therapy (HRT), the diagnosis of breast cancer, and the clinical outcome of women who develop breast cancer in the setting of HRT continue to present important challenges for health professionals and the public. The general impression in the medical community is that breast cancer is diagnosed more frequently after prolonged HRT; however, whether a causative relationship exists between HRT and breast cancer remains uncertain. Despite the increase in breast cancer diagnosis, clinical outcome of the disease appears favorable for women who develop breast cancer after HRT; HRT users tend to present with more localized tumors that exhibit favorable histology, and overall death from breast cancer appears reduced. The history of breast cancer constitutes a contraindication for HRT as a general rule, based on the concern that HRT may activate dormant cancer cells. Direct evidence for this practice is very limited; available studies are small and mostly retrospective, but do not indicate an adverse effect of HRT on breast cancer recurrence. In reaching a clinical decision regarding HRT, the expected benefits (largely improvement of menopausal symptoms) must be weighed against the potential risks of HRT (especially breast cancer) for each individual woman.

Role of preoperative ultrasonography in the surgical management of patients with thyroid cancer.

BACKGROUND: Cervical recurrence occurs in up to 30% of patients with differentiated thyroid carcinoma. We retrospectively compared preoperative transcutaneous ultrasonography and physical examination (PE) results in the detection of local-regional metastases (lymph node and soft tissue) in patients with thyroid cancer. METHODS: Data were collected retrospectively from the medical records of patients with thyroid carcinoma who underwent preoperative ultrasonography. Patients were divided into 3 groups: group 1, those undergoing primary thyroid/neck surgery; group 2, those undergoing reoperation for persistent disease; and group 3, those undergoing reoperation for recurrent thyroid carcinoma. For each group, we recorded the frequencies with which ultrasonography detected disease in a neck compartment (central or lateral) that was normal on PE. RESULTS: Two hundred twelve patients underwent operation for primary, persistent, or recurrent papillary (n=130), medullary (n=61), or follicular/Hürthle cell (n=21) carcinoma. Ultrasonography detected additional sites of metastatic disease not appreciated on PE in 21 (20%) of 107 group 1 patients, 9 (32%) of 28 group 2 patients, and 52 (68%) of 77 group 3 patients. The surgical procedure performed was altered by the information obtained from preoperative ultrasonography in 82 (39%) of the 212 patients. Of the 107 group 1 patients, cervical recurrence has been detected in only 6 (6%) at a median follow-up of 36 months, in spite of 67 (63%) having tumors larger than 2 cm or lymph node metastases. CONCLUSIONS: Preoperative high-quality ultrasonography detected lymph node or soft-tissue metastases in neck compartments believed to be uninvolved by PE in 39% of patients. Ultrasound findings altered the operative procedure in these patients, facilitating complete resection of disease and potentially minimizing local-regional recurrence.

Improving the yield of preoperative parathyroid localization: technetium Tc 99m-sestamibi imaging after thyroid suppression.

BACKGROUND: Preoperative localization is essential for successful directed, minimally invasive or reoperative parathyroidectomy. Standard technetium Tc 99m-sestamibi imaging is the most sensitive modality for localization. We reviewed our experience with (99m)Tc-sestamibi imaging and specifically investigated the effect of thyroid suppression on repeat imaging of patients who had initially nonlocalizing scans. METHODS: . The records of patients who underwent (99m)Tc-sestamibi imaging during evaluation for primary hyperparathyroidism were reviewed. A subset of patients with initially nonlocalizing scans underwent thyroid suppression with either thyroxin or liothyronine and then had their scans repeated. RESULTS: Ninety-nine patients with primary hyperparathyroidism underwent (99m)Tc-sestamibi imaging followed by parathyroidectomy (initial operation, 78; reoperation, 21). Successful parathyroid localization was obtained on standard imaging in 67 patients. Fourteen of 32 patients who had nonlocalizing (99m)Tc-sestamibi imaging studies underwent an additional scan after thyroid suppression. In 10 of 14 patients (71%), repeat (99m)Tc-sestamibi imaging after thyroid suppression successfully localized abnormal parathyroid tissue. CONCLUSIONS: Thyroid suppression may improve the yield of (99m)Tc-sestamibi imaging in patients with hyperparathyroidism who have an initially nonlocalizing study. This diagnostic strategy may be helpful in patients motivated to undergo a directed, minimally invasive operation, as well as in the evaluation of patients for reoperative parathyroidectomy.

Triiodothyronine supplementation for hypothalamic obesity.

Patients with suprasellar lesions develop profound hypothalamic obesity and listlessness with no effective treatment. We added triiodothyronine (T(3)) supplementation in 3 such patients and present their response. All had previous nutritional counseling without benefit. All were treated for diabetes insipidus (DI) and hypopituitarism; serum free thyroxine (T(4)) level was normal. A 24-year-old woman (pineal tumor and astrocytoma) had weight gain (4.7 kg/yr for 3 years), cold intolerance, fatigue, dry skin, and constipation; after T(3), she lost 14 kg over 27 months and reported overall improvement. Her bone mineral density also improved. A 10.6-year-old boy (optic glioma) was gaining 6 kg/yr for 4 years; after T(3) supplement, he lost 4.3 kg over 11 months. A 12-year-old girl (mixed germ cell tumor) had weight gain (8.3 kg/yr for 3 years) and listlessness; after T(3), she lost 8.1 kg over 16 months and had improved alertness. All patients were asymptomatic despite supraphysiologic T(3) levels. We suggest that T(3) may serve as a simple and effective supplement, which can promote weight loss and improve the well being of these patients with hypothalamic obesity.