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Background: Very young breast cancer at 35 years or younger is rare and tends to be aggressive. The management of very young breast cancer should be equally aggressive, and early diagnosis is critical. We hypothesized that socioeconomic status (SES) is associated with how very young breast cancer (VYBC) patients are diagnosed and treated in our community. Methods: We retrospectively analyzed a database for invasive breast cancer or ductal carcinoma in-situ (DCIS) diagnosed ≤35 years old (VYBC) from both a government-run county hospital (CH) and a neighboring private hospital (PH) for 82 months. Inclusion criteria composed of female patients diagnosed with invasive breast cancer and DCIS at ≤35 years of age at diagnosis were examined in our analysis. Patient diagnoses and severity, treatment, and outcomes were compared between the two facilities. The methods are consistent with the STROCSS guidelines. Results: The incidence of VYBC was a significantly higher percentage of new breast cancers diagnosed in the CH at 5.4% as compared to the PH at 1.9% (OR: 2.94; p < 0.001). CH had a significantly higher proportion of Medicaid payers (73% CH vs. 21% PH, p < 0.001). Mean time from patient's initial symptoms until a tissue diagnosis was significantly longer at the county hospital at 9.6 months vs 2.1 months at the private hospital (p < 0.01). Stage I tumors were only found in 13% of county hospital patients versus 67% at the private hospital (p < 0.001). Approximately 63% of the patients at the government-run county facility had a relative delay in diagnosis, contributing to higher stage at presentation. The rate of mastectomy surgery was high at both institutions: CH 83% vs. PH 76% (p = NS). Discussion: The results of our study suggest a significant healthcare difference between county and private hospital systems among very young breast cancer patients. Patients at the county hospital system had a longer delay to diagnosis leading to worse outcomes. This difference may be a reflection of barriers in health literacy or access to medical care.
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INTRODUCTION: The COVID-19 pandemic decreased in-person didactic sessions in our general surgery residency. We piloted a program to develop online tutorials posted to a YouTube channel designed to prepare our residents for the ABSITE examination. METHODS: Study participants were General Surgery residents in our five-year ACGME accredited general surgery residency program who took the ABSITE in-service exam between 2019 and 2021. We compared raw scores and overall programmatic percentile scores for three academic years (2018-2019, 2019-2020, and 2020-2021). RESULTS: From academic year 2018-2019 to 2019-2020, before introducing the YouTube teaching platform, average raw scores and percentile scores among our general surgery residents remained unchanged (raw scores 64 - 65% (p = ns), percentile scores 39 to 37% (p = ns)). However, raw ABSITE scores increased significantly after introducing the YouTube teaching platform. A one-way ANOVA showed a statistically significant difference in raw ABSITE scores for AY 2020-2021 compared to the two prior years (F(2, 30) = [1.193], P < 0.01). Mean percentile ranking scores (program-wide) increased 15% from AY 2018-2019 to AY 2020-2021 (Figure1). CONCLUSION: Teaching via an online platform such as YouTube allows residents to review material at their own pace and schedule. It is other our hypothesis is that the online YouTube material presented in the Ventura Surgery School channel contributed, in part, to this relative improvement compared to resident peers in other institutions. Teaching via an online platform could be a valuable tool to educate surgical learners in our ever-changing teaching environment.
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COVID-19 , Cirurgia Geral , Internato e Residência , Mídias Sociais , COVID-19/epidemiologia , Competência Clínica , Educação de Pós-Graduação em Medicina , Avaliação Educacional , Cirurgia Geral/educação , Humanos , PandemiasRESUMO
INTRODUCTION: Dedifferentiated liposarcoma is a rare, highly malignant, rapidly recurring tumor. When found in the spermatic cord, this tumor may deceptively present as an inguinal hernia, and due to its rarity, may not be readily identified. PRESENTATION OF CASE: We report a case of a 46-year old man with dedifferentiated liposarcoma presenting as a slow-growing right inguinal bulge. His surgical management included ipsilateral orchiectomy and excision with wide margins. He received adjuvant focused radiation therapy. CONCLUSION: Liposarcoma of the Spermatic Cord is a rare and highly malignant tumor that rapidly recurs. Therefore, a high degree of suspicion, regular surveillance and imaging are justified.
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We report a case of a patient who presented with biliary colic while in Tijuana, Mexico. Laparoscopic cholecystectomy was attempted but abandoned and only a biopsy of the gallbladder was performed with pathologist reporting gallbladder adenocarcinoma. Upon return to the United States, extensive evaluation was undertaken including imaging, biopsy, and ultimately two separate exploratory surgeries revealing no neoplasm. Only at the second surgical exploration did we discover a benign cholecystoduodenal fistula successfully treated with completion fenestration cholecystectomy, pyloric exclusion, loop gastrojejunostomy, and duodenostomy tube through the gallbladder remnant into the fistula itself. This is a unique surgical treatment of a rare problem made even more confusing by an erroneous pathology report from another country.
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INTRODUCTION: Many breast cancer patients remove their contralateral breast unaffected by cancer, commonly referred to as contralateral prophylactic mastectomy (CPM). CPM has been extensively studied and does not improve survival in patients without deleterious genetic mutation or lobular histology. Despite these limited indications, CPM rates have increased recently. We observed CPM trends in our county safety-net hospital. METHODS: We performed a retrospective review of women treated for breast cancer who underwent bilateral mastectomy in our institution. RESULTS: We analyzed 100 CPMs: 51% (n = 51) had bilateral breast MRI before CPM, 54% (n = 54) had genetic testing prior to CPM, and 56% (n = 30) had a deleterious genetic result. Another 12% (n = 12) had lobular histology as the primary determinant for CPM. Of patients without genetic mutation or lobular histology, 13% (n = 13) had suspicious MRI findings in the CPM side that drove the decision for CPM. Forty-seven percent (n = 47) had reconstructive surgery documented. CONCLUSION: Only 42% (n = 42) of patients who underwent CPM had a documented justifiable medical reason. In the future, physician and patient education may decrease unindicated CPM. We also found that 13% of women without a medical indication chose CPM based on suspicious MRI findings, while only 8% of these MRI findings were malignant. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266.
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Neoplasias da Mama , Mastectomia Profilática , Mama , Neoplasias da Mama/genética , Neoplasias da Mama/prevenção & controle , Neoplasias da Mama/cirurgia , Feminino , Humanos , Mastectomia , Estudos RetrospectivosRESUMO
Herein we describe a case report of a patient with elevated liver enzymes, leukocytosis, anemia and fevers after cholecystectomy surgery done for presumed acute cholecystitis. Numerous post-surgical tests showed no acute surgical complications to account for the laboratory abnormalities. Due to systemic symptoms of joint pain and the chronicity of the symptoms, a liver biopsy was recommended by the gastroenterology service to rule out infectious or auto-immune causes. After the liver biopsy, the patient was diagnosed with intravascular large B-cell lymphoma (IVLBCL), a subset of diffuse large B-cell lymphoma. After a thorough lymphoma evaluation, the IVLBCL was found to be isolated to the liver and treated successfully with chemotherapy. This is only the second case report in the literature of this entity.
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BACKGROUND: For patients with melanoma, if there has been no recurrence of disease 10 years after initial treatment, additional disease is believed to be very unlikely. However, such late recurrences are known to occur. The frequency of this phenomenon and its clinical significance are not well characterized due to the difficulty in obtaining relevant data. We examined a large, mature, institutional database to evaluate late recurrence. STUDY DESIGN: The late recurrence cohort was defined as having a disease-free interval of 10 or more years after potentially curative treatment and was compared with an early recurrence cohort recurring within 3 years. Actuarial late recurrence frequency and factors associated with late recurrence were examined. Post-recurrence overall and melanoma-specific survival and prognostic variables were analyzed. RESULTS: Among all patients, 408 exhibited late recurrence (mean disease-free interval 15.7 years). For patients who received primary treatment at our institution with 10 or more years follow-up, 327 of 4,731 (6.9%) showed late recurrence. On an actuarial basis, late recurrence rates were 6.8% and 11.3% at 15 and 20 years, respectively, for those with no recurrence at 10 years. Late recurrence was associated with both tumor (thin, non-ulcerated, non-head/neck, node negative) and patient (younger age, less male predominant) characteristics. Multivariate analysis confirmed younger age, thinner and node negative tumors in the late recurrence group. Late recurrences were more likely to be distant, but were associated with better post-recurrence survival on univariate and multivariate analyses. CONCLUSIONS: Late melanoma recurrence is not rare. It occurs more frequently in certain clinical groups and is associated with improved post-recurrence survival.
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Melanoma/secundário , Recidiva Local de Neoplasia , Neoplasias Cutâneas/secundário , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Masculino , Melanoma/etiologia , Melanoma/mortalidade , Melanoma/terapia , Pessoa de Meia-Idade , Análise Multivariada , Recidiva Local de Neoplasia/etiologia , Recidiva Local de Neoplasia/mortalidade , Fatores de Risco , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Análise de Sobrevida , Adulto JovemRESUMO
KEY CLINICAL MESSAGE: We present a case of hepatocellular carcinoma located within the pancreas. These tumors occur in the body and tail of the pancreas, with a male predominance, and at a younger age. Tumors with pure hepatocellular histopathology have better survival and recurrence rates and should be offered surgical therapy if possible.
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BACKGROUND: Preoperative assessment of the nipple-areolar complex (NAC) is invaluable when considering nipple-sparing mastectomy. Our hypothesis is that breast magnetic resonance imaging (MRI) may predict involvement of the NAC with tumor. METHODS: Clinical, histopathologic, and imaging data were compiled for patients who underwent preoperative breast MRI followed by mastectomy or nipple-sparing mastectomy for malignancy between 2006 and 2009. Blinded rereview of all MRI studies was performed by a breast MRI imager and compared to initial MRI findings. Multivariate analysis identified variables predicting NAC involvement with tumor. RESULTS: Of 77 breasts, 18 (23 %) had tumor involving or within 1 cm of the NAC. The sensitivity of detecting histopathologically confirmed NAC involvement was 61 % with history and/or physical examination, and 56 % with MRI. Univariate analysis identified the following variables as significant for NAC involvement: large tumors near the nipple on preoperative MRI, node-positive disease, invasive lobular carcinoma, advanced histopathologic T stage, and neoadjuvant chemotherapy. On multivariate analysis, only tumor size >2 cm and distance from tumor edge to the NAC <2 cm on MRI maintained significance. Pearson correlation coefficient for MRI size compared to histopathologic size was 0.53 (P < 0.0001). CONCLUSIONS: MRI is not superior to thorough clinical evaluation for predicting tumor in or near the NAC. However, MRI-measured tumor size and distance from the NAC are correlated with increased risk of NAC involvement. The combination of preoperative history and physical examination, tumor characteristics, and breast MRI can aid the surgeon in predicting a tumor-involved nipple more than any single modality alone.
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Neoplasias da Mama/patologia , Carcinoma Ductal de Mama/patologia , Carcinoma Intraductal não Infiltrante/patologia , Carcinoma Lobular/patologia , Imageamento por Ressonância Magnética , Mamilos/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/cirurgia , Carcinoma Ductal de Mama/cirurgia , Carcinoma Intraductal não Infiltrante/cirurgia , Carcinoma Lobular/cirurgia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Mamilos/cirurgia , Cuidados Pré-Operatórios , Prognóstico , Estudos ProspectivosRESUMO
Accurate determination of the size or extent of ductal carcinoma in situ (DCIS) by imaging is uncertain, and incomplete resection of tumor results in involved margins in up to 81% of cases. This study examined the accuracy of magnetic resonance imaging (MRI) for assessment of DCIS size, and evaluated the effect of preoperative breast MRI on achievement of tumor-free surgical margins after breast-conserving surgery (BCS). One-hundred and fifty-eight female patients with DCIS were identified from a prospective database: 60 patients (62 cases) had preoperative breast MRI, and 98 patients did not have MRI. The accuracy of tumor size assessed by MRI was determined by comparison with histopathologic size. All patients underwent BCS initially. The rate of involved margins after resection was compared in MRI and no-MRI groups. The overall correlation between MRI size and histopathologic size was high (p < 0.0001). MRI assessment of size was significantly more accurate when DCIS was high grade (p < 0.0001) or intermediate grade (p = 0.005) versus low grade (p = 0.187). The rate of tumor-involved margins was not significantly different in MRI and no-MRI groups (30.7% and 24.7%, respectively; p = 0.414). The rate of mastectomy was significantly higher in the MRI group than the no-MRI group (17.7% versus 4.1%; p = 0.004). These findings indicate that MRI can detect DCIS, especially when lesions are high or intermediate grade, but that MRI does not accurately predict the size of DCIS. In this study, MRI did not improve the surgeon's ability to achieve clear margins following BCS.
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Neoplasias da Mama/diagnóstico , Neoplasias da Mama/patologia , Carcinoma Intraductal não Infiltrante/diagnóstico , Carcinoma Intraductal não Infiltrante/cirurgia , Imageamento por Ressonância Magnética/métodos , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/cirurgia , Carcinoma Intraductal não Infiltrante/patologia , Feminino , Humanos , Mastectomia Segmentar , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Cuidados Pré-Operatórios , Estudos Prospectivos , Taxa de SobrevidaRESUMO
BACKGROUND: We postulated that a surgical shunt between the portal and hepatic veins (portohepatic shunt) could improve the function and regeneration of the liver remnant after extensive hepatic resection. Using a canine model, we designed a survival study to test the feasibility of 90% hepatectomy with a portohepatic shunt. Of eight mixed-hound canines, it was necessary to sacrifice two animals for postmortem study of hepatic anatomy; we used the results to design a procedure for removing all of the liver except portions of two lobes. We tested this 90% hepatectomy procedure under general anesthesia in the remaining animals, with (N=3) or without (N=3) a portohepatic shunt. We monitored animals postoperatively with serial liver biochemical testing, and sacrificed on postoperative day 48. Assessment of liver regeneration was by weight after complete postmortem hepatectomy. Extended hepatectomy removed four complete lobes and portions of the right lateral and caudate lobes. For the portohepatic shunt, we anastomosed the left portal vein branch to the left hepatic vein branch. All procedures were performed successfully. One no-shunt animal expired on the second postoperative day. We thus confirmed the feasibility of 90% hepatectomy with portohepatic shunt in the canine model. If the efficacy of shunting is validated in a larger study population, it may increase the indications for extensive resection in patients with advanced hepatic malignancy.
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Hepatectomia/métodos , Fígado/cirurgia , Animais , CãesRESUMO
BACKGROUND: Basal-like breast cancer (BLBC) has a poor prognosis and is often identified by the triple-negative phenotype (TNP) and/or basal cytokeratins (CKs). Overexpression of mRNA for forkhead box C1 (FOXC1) transcription factor was recently identified as a pivotal prognostic biomarker of BLBC. We investigated the prognostic value of FOXC1 protein expression in invasive breast cancer and compared its prognostic significance to that of TNP and basal CKs. METHODS: Archived TNP specimens of primary invasive ductal breast cancer from 759 patients were examined by immunohistochemical staining for FOXC1, CK5/6, and CK14; prognostic significance was assessed using multivariate analyses. In addition, the impact of adding FOXC1 versus basal CKs to TNP-based BLBC assessment was assessed. RESULTS: FOXC1 protein expression was a significant predictor of overall survival on univariate (hazard ratio [HR] 3.364 95% confidence interval [CI] 1.758-6.438, P = 0.0002) and multivariate (HR 3.389 95% CI 1.928-7.645, P = 0.0001) analyses, despite its correlation with younger age (P = 0.0003). Interestingly, nodal status was not significant on multivariate analysis when FOXC1 expression status was included in the analysis. BLBC defined by TNP plus FOXC1 demonstrated superior prognostic relevance compared to BLBC defined by TNP or TNP plus basal CKs. CONCLUSIONS: Immunohistochemical detection of FOXC1 expression in TNP invasive breast cancer is an independent prognostic indicator that is superior to conventional immunohistochemical surrogates of BLBC. Prospective validation is warranted to further define the diagnostic, prognostic, and predictive utility of FOXC1 in breast cancer management and clinical trial design.
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Biomarcadores Tumorais/metabolismo , Neoplasias da Mama/metabolismo , Carcinoma Basocelular/metabolismo , Carcinoma Ductal de Mama/metabolismo , Fatores de Transcrição Forkhead/metabolismo , Neoplasias da Mama/mortalidade , Neoplasias da Mama/patologia , Carcinoma Basocelular/mortalidade , Carcinoma Basocelular/patologia , Carcinoma Ductal de Mama/mortalidade , Carcinoma Ductal de Mama/patologia , Feminino , Seguimentos , Humanos , Técnicas Imunoenzimáticas , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Estudos Prospectivos , Receptor ErbB-2/metabolismo , Receptores de Estrogênio/metabolismo , Receptores de Progesterona/metabolismo , Taxa de SobrevidaRESUMO
BACKGROUND: Although most melanomas on the distal lower extremity drain exclusively to inguinal lymph nodes, a small percentage (<5%) drain to interval nodes in the popliteal basin. We investigated a possible relationship between tumor-draining popliteal and inguinal nodes in patients with lower-extremity melanoma. STUDY DESIGN: We queried our melanoma database to identify patients who underwent sentinel node biopsy (SNB) for an infrapopliteal melanoma. Patterns of nodal drainage and nodal metastasis were analyzed. RESULTS: Of 461 patients who underwent SNB for a primary infrapopliteal melanoma, 15 (3.2%) had drainage to the popliteal basin. Thirteen melanomas were on the posterior leg and foot, and 2 were on the anterior lower leg. Mean Breslow thickness was 2.4 mm. All 15 patients with popliteal drainage also had inguinal drainage and therefore underwent concurrent inguinal and popliteal SNB. The average number of popliteal sentinel nodes was 1.4 (range 1 to 3). Eight patients (53%) had a tumor-positive popliteal sentinel node, and 6 of the 8 underwent completion popliteal lymphadenectomy. Four of the 8 patients (50%) also had tumor-positive inguinal sentinel nodes; all underwent complete inguinal lymphadenectomy. We also identified 9 additional patients who underwent SNB for locoregional recurrent melanomas of the infrapopliteal leg. Three (33%) of these patients had concurrent inguinal and popliteal SNB, with 1 isolated tumor-positive popliteal node found. CONCLUSIONS: In our series, a high percentage of popliteal sentinel lymph nodes contained metastases, and these patients frequently also had inguinal metastases. In our patients, all inguinal metastases were associated with concomitant popliteal metastases. Although it is anatomically separate, the inguinal basin appears to be a functional extension of the popliteal basin.
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Melanoma/secundário , Melanoma/cirurgia , Biópsia de Linfonodo Sentinela , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgia , Adulto , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Humanos , Canal Inguinal , Perna (Membro) , Masculino , Melanoma/mortalidade , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Cutâneas/mortalidade , Adulto JovemRESUMO
BACKGROUND: The efficacy of surgery for invasive mucinous neoplasms is unclear. We examined the natural history of invasive mucinous cystic neoplasms (MCN) and invasive intraductal papillary mucinous neoplasms (IPMN) in patients who underwent pancreatic resection. METHODS: The Surveillance, Epidemiology, and End Results (SEER) database (1996-2006) was queried for cases of resected invasive MCN and IPMN. Demographics, tumor characteristics, and overall survival were examined using log-rank analysis and multivariate Cox regression model. RESULTS: Of 185 MCN cases and 641 IPMN cases, 73% and 48%, respectively, were women (P < 0.0001). Most (73%) IPMN were in the head of the pancreas; most (64%) MCN were in the tail/body (P < 0.0001). Lymph node metastasis was more common for IPMN than MCN (46% vs. 24%, P < 0.0001). Overall survival after resection was better for patients with stage I MCN vs. stage I IPMN (P = 0.0005), and it was better for patients with node-negative MCN vs. node-negative IPMN (P = 0.0061). There was no significant difference in survival of patients with stage IIA MCN vs. stage IIA IPMN (P = 0.5964), stage IIB MCN vs. stage IIB IPMN (P = 0.2262), or node-positive MCN vs. node-positive IPMN (P = 0.2263). Age older than 65 years (hazards ratio (HR) 1.71, P = 0.0046), high tumor grade (HR 2.68, P < 0.0001), higher T stage (HR 2.11, P < 0.0001), and IPMN histology (HR 1.90, P = 0.0040) predicted worse outcome in node-negative patients. CONCLUSIONS: Our findings suggest that survival is better after resection of invasive MCN versus invasive IPMN when disease is localized within the pancreas, but this difference disappears in the presence of nodal metastasis or extrapancreatic extension.
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Adenocarcinoma Mucinoso/mortalidade , Carcinoma Ductal Pancreático/mortalidade , Carcinoma Papilar/mortalidade , Pancreatectomia , Neoplasias Pancreáticas/mortalidade , Adenocarcinoma Mucinoso/patologia , Adenocarcinoma Mucinoso/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Ductal Pancreático/patologia , Carcinoma Ductal Pancreático/cirurgia , Carcinoma Papilar/patologia , Carcinoma Papilar/cirurgia , Criança , Estudos de Coortes , Feminino , Humanos , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica , Estadiamento de Neoplasias , Neoplasias Pancreáticas/patologia , Neoplasias Pancreáticas/cirurgia , Estudos Retrospectivos , Programa de SEER , Taxa de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
The purpose of this study was to identify patients at risk for hypocalcemia after parathyroid surgery. The charts of 99 patients who underwent parathyroid surgery over a 2-year period using a rapid intraoperative parathyroid hormone (IOPTH) assay were retrospectively reviewed. Data for patient demographics, preoperative parathyroid hormone and calcium levels, IOPTH levels, and postoperative calcium levels were reviewed, and trends were analyzed for patients who had postoperative hypocalcemia. Of the 99 patients, 91 had one gland excised and 8 had two glands excised. Preoperative calcium levels ranged from 8.9 to 15.6 mg/dL. Sixty-seven of the patients had a >50% drop in IOPTH at 5 minutes, and 82 had a >50% drop in IOPTH by 10 minutes. Twelve patients had early hypocalcemia. Parathyroid weight and surgery type were not significant factors in predicting postoperative hypocalcemia. A drop of >80% in IOPTH at 10 minutes was a significant factor for postoperative hypocalcemia (P = 0.02). In addition, having normal or only minimally elevated preoperative calcium was an independent risk factor for postoperative hypocalcemia (P = 0.05). Our study has shown that postoperative calcium supplementation and in-hospital monitoring should be considered in patients with a preoperative calcium level in the normal range and with an IOPTH that has dropped by >80%.
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Melanoma is a prevalent and deadly disease with limited therapeutic options. Current prognostic factors are unable to adequately guide treatment. Circulating tumor cells are a disease-specific factor that can be used as a prognostic variable to guide therapy. Most research to date has focused on identification of circulating tumor cells using various methods, including polymerase chain reaction. These techniques, however, have poor sensitivity and variable specificity and predictive significance. A recently developed technology to identify circulating tumor cells is the CellSearch system. This system uses immunomagnetic cell labeling and digital microscopy. This technology may provide an alternative method to identify circulating tumor cells in patients with advanced-stage melanoma and function as a prognostic factor. We review the literature on circulating tumor cells in melanoma and present data collected at our institution using the CellSearch system in nine patients with stage III or IV melanoma.
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Acute gastric dilation leading to ischemia of the stomach is an under-diagnosed and potentially fatal event. Multiple etiologies can lead to this condition, and all physicians should be aware of it. Without proper and timely diagnosis and treatment, gastric perforation, hemorrhage, and other serious complications can occur. We report a case of acute gastric dilation and ischemia secondary to small bowel obstruction. We also review the world literature and discuss the etiology, diagnosis, and management of this condition.