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Neurofibromatosis type 1: a case highlighting pulmonary and other rare clinical manifestations.

Tue, 09/18/2018 - 12:31
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Neurofibromatosis type 1: a case highlighting pulmonary and other rare clinical manifestations.

BMJ Case Rep. 2018 Jan 31;2018:

Authors: Nguyen KA, Elnaggar M, Gallant NM, Tanios M

Abstract
Neurofibromatosis type 1 (NF1)-related lung disease is a rare but increasingly recognised, high morbidity associated feature of the condition. We present a 48-year-old male patient with NF1, who was initially admitted for a subarachnoid haemorrhage requiring aneurysmal coil embolisation. During his recovery, he developed a left-sided pneumothorax requiring chest tube placement followed by concerns for re-expansion pulmonary oedema requiring intubation. Subsequently, the patient also developed a right-sided pneumothorax requiring additional chest tube placement but did not develop right-sided pulmonary oedema. During his hospitalisation, the patient also exemplified other important NF1-related pathophysiology including pheochromocytoma, cerebrovascular abnormalities and cardiovascular manifestations. Due to his multiple comorbidities and poor prognosis, we held a goals of care discussion with the patient's mother, and with her agreement, the patient underwent compassionate withdrawal of artificial life support.

PMID: 29386211 [PubMed - indexed for MEDLINE]

The Safety and Efficacy of Combination Therapy of Sorafenib and Radiotherapy for Advanced Hepatocellular Carcinoma: A Retrospective Study.

Tue, 09/18/2018 - 12:31
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The Safety and Efficacy of Combination Therapy of Sorafenib and Radiotherapy for Advanced Hepatocellular Carcinoma: A Retrospective Study.

Intern Med. 2018 May 15;57(10):1345-1353

Authors: Wada Y, Takami Y, Matsushima H, Tateishi M, Ryu T, Yoshitomi M, Matsumura T, Saitsu H

Abstract
Objective Sorafenib is a standard therapy for advanced hepatocellular carcinoma (HCC), whereas radiotherapy is effective for local control of extrahepatic spread (EHS) or macrovascular invasion (MVI). This study investigated the safety and efficacy of this combined therapy to treat advanced HCC. Methods This retrospective study reviewed 62 patients with advanced-stage HCC with EHS or MVI who received sorafenib therapy, excluding the patients with only lung metastases. Results Of the 62 patients, 15 were treated using the combined therapy of sorafenib and radiotherapy (group RS), and 47 were treated with sorafenib monotherapy (group S). In group RS, patients were treated using three-dimensional conformal radiotherapy with a total irradiation dose of 30-60 Gy (median, 50 Gy). Irradiation was targeted at the bone, lymph nodes, adrenal gland, and MVI in 6, 5, 1, and 4 patients, respectively. The overall incidence of adverse events was 93.3% in group RS and 91.5% in group S (p=N.S.). Incidences of thrombocytopenia, leukopenia, and skin reaction were significantly higher in group RS (73.3%, 40.0%, and 66.7%, respectively) than in group S (36.2%, 10.6%, and 27.7%, respectively, p=0.02, 0.02, and <0.01, respectively). The incidence of severe adverse events, however, was comparable in the 2 groups: 20% in group RS and 19.2% in group S. The median progression-free survival (PFS) of EHS or MVI, PFS of whole lesions, and overall survival were longer in group RS (13.5, 10.6, and 31.2 months, respectively) than in group S (3.3, 3.5, and 12.1 months, respectively) (p<0.01 for all). Conclusion Sorafenib in combination with radiotherapy is a feasible and tolerable treatment option for advanced HCC.

PMID: 29279513 [PubMed - indexed for MEDLINE]

Decreased Serum Adiponectin Level during Catecholamine Crisis in an Obese Patient with Pheochromocytoma.

Tue, 09/18/2018 - 12:31
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Decreased Serum Adiponectin Level during Catecholamine Crisis in an Obese Patient with Pheochromocytoma.

Intern Med. 2018 May 01;57(9):1253-1257

Authors: Okauchi Y, Ishibashi C, Shu K, Adachi S, Mineo I

Abstract
We herein report the case of a 37-year-old man with both pheochromocytoma and visceral fat accumulation and describe the sequential changes in his adiponectin levels throughout the clinical course from catecholamine crisis until the follow-up for adrenalectomy. His adiponectin level decreased during catecholamine crisis and increased after adrenalectomy. However, his adiponectin level decreased again at two years postoperatively when his visceral fat area greatly increased. This case suggests that catecholamines and visceral fat volume may affect adiponectin metabolism in subjects with pheochromocytoma, which may precipitate cardiovascular complications in this endocrine disease.

PMID: 29279477 [PubMed - indexed for MEDLINE]

adrenal tumor; +31 new citations

Tue, 09/11/2018 - 12:25

31 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

adrenal tumor

These pubmed results were generated on 2018/09/11

PubMed comprises more than millions of citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.

Malignant peripheral nerve sheath tumor originating from the adrenal gland in a dog.

Tue, 09/04/2018 - 09:58
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Malignant peripheral nerve sheath tumor originating from the adrenal gland in a dog.

J Vet Med Sci. 2018 Sep 03;:

Authors: Ichikawa M, Suzuki S, Tei M, Nibe K, Uchida K, Ono K, Hirao H

Abstract
A large abdominal mass was found in a dog. Histopathologically, the surface of the mass was covered with compressed adrenal gland tissue. The neoplastic cells showed typical features of malignant peripheral nerve sheath tumor (MPNST), including Antoni type A and type B pattern, and nuclear palisading. Immunohistochemically, these cells were positive for S100 protein, nerve growth factor receptor, nestin and claudin-1. The dog was euthanized because of the developing multiple metastatic lesions. The metastatic cells showed quite similar histopathological and immunohistochemical features as those in the original tumor. Although MPNST can develop at many body sites, this is the first report of MPNST originating from the adrenal gland in a dog.

PMID: 30175755 [PubMed - as supplied by publisher]

Posterior retroperitoneoscopic adrenalectomy-Case series.

Tue, 09/04/2018 - 09:58
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Posterior retroperitoneoscopic adrenalectomy-Case series.

Int J Surg Case Rep. 2018 Aug 28;51:174-177

Authors: Costa Almeida CE, Caroço T, Silva MA, Albano MN, Louro JM, Carvalho LF, Costa Almeida CM

Abstract
INTRODUCTION: Posterior retroperitoneoscopic adrenalectomy has advantages over transperitoneal technique. However many surgeons prefer the transperitoneal technique because they get a familiar and wider working space.
MATERIAL AND METHODS: A retrospective analysis of the first 10 patients submitted to posterior retroperitoneoscopic adrenalectomy was conducted. Data collected included: diagnosis, size, operation time, blood loss, conversion rate, morbidity and mortality, in-hospital length of stay. Compare our outcomes with worldwide bigger series, and take conclusions on the feasibility of the technique was the objective.
RESULTS: We included 2 pheochromocytomas, 1 giant cystic pheochromocytoma, 4 Conn's, 2 Cushing's, 1 non-functioning tumor with 4 cm. Mean operation time was 46,7 min for lesions ranging from 1,8 to 14 cm. Blood loss was negligible. One patient (10%) was converted to laparotomy because of a past clinical history of dorsal and lumbar trauma. No morbidity and no mortality. Mean hospital length of stay was 2,2 days.
DISCUSSION: Mean operation time found in bigger series published in worldwide literature is 40-105,6 min. Complication rate reported ranges from 0 to 14,4%. No mortality has been ever reported. Blood loss reported in other series is 10-50 ml. The data found in our study matches other studies data. Since the same surgeon who had never performed the technique before operated all patients, makes us believe the technique is safe and feasible.
CONCLUSION: Posterior retroperitoneoscopic adrenalectomy has a small learning curve. It is technically safe and feasible. More patients will be collected to validate these results.

PMID: 30173077 [PubMed - as supplied by publisher]

Tubulo-papillary adrenocortical adenoma in a patient with familial adenomatous polyposis: A morphologic, ultrastructural and molecular study.

Tue, 09/04/2018 - 09:58
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Tubulo-papillary adrenocortical adenoma in a patient with familial adenomatous polyposis: A morphologic, ultrastructural and molecular study.

Hum Pathol. 2018 Aug 30;:

Authors: Huang H, Chang J, Rosati S, Geurts J, Mackinnon AC

Abstract
Patients with Familial Adenomatous Polyposis (FAP) have a higher incidence for developing adrenal neoplasms, most of which are non-functioning with conventional histologic appearance. We report a patient with a history of multiple colon polyps who developed an adrenocortical adenoma with unusual morphology. The tumor showed a tubulo-papillary architecture and plasmacytoid cytomorphology that were distinct from conventional adrenocortical adenomas. β-catenin stain showed aberrant nuclear positivity in the tumor, suggesting an altered β-catenin related pathway. The unusual morphology prompted molecular characterization, and sequencing demonstrated the patient to be germline heterozygous for a 5-base pair APC deletion at codon 1309 with loss of heterozygosity (LOH) in the tumor. Our study provides further evidence of genetic predisposition to extra-intestinal tumors in the FAP population.

PMID: 30172912 [PubMed - as supplied by publisher]

Organ-specific response to nivolumab in patients with non-small cell lung cancer (NSCLC).

Tue, 09/04/2018 - 09:58
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Organ-specific response to nivolumab in patients with non-small cell lung cancer (NSCLC).

Cancer Immunol Immunother. 2018 Aug 31;:

Authors: Schmid S, Diem S, Li Q, Krapf M, Flatz L, Leschka S, Desbiolles L, Klingbiel D, Jochum W, Früh M

Abstract
BACKGROUND: Response to immune checkpoint inhibitors depends on tumor intrinsic properties and also on host factors in the tumour microenvironment including the presence of immune cells (IC). We hypothesized that nivolumab efficacy varies across different metastatic sites.
METHODS: We retrospectively analyzed computed tomography scans of patients with metastatic non-small cell lung carcinoma (NSCLC) receiving nivolumab. RECIST 1.1 criteria were applied to assess the overall response rate (ORR) and organ-specific response rate (OSRR).
RESULTS: We analyzed 52 patients including 44% females, 58% adenocarcinoma and 8% never smokers. Involved organs had target-lesions in the lung (42%), liver (25%), lymph nodes (56%) and soft tissue (13%) and non-target lesions in the bones (23%). ORR and disease control rate (DCR) were 20% and 45%, respectively. Median overall survival, progression-free survival and duration of response were 11.9, 2.3 and 10.3 months. OSRR and organ-specific DCR (OSDCR) were 28% and 90% in lymph nodes, 8% and 54 in the liver, and 9% and 55% in lung metastases. Nine out of 12 patients with bone metastases had progressive lesions. The cumulative incidence probability of organ-specific progression at 6 months was 14% in lymph nodes, 42% in the liver, 36% in lung metastases and 26% in the primary tumor, 29% in soft tissue and 33% in adrenal metastases.
CONCLUSION: In conclusion, the efficacy of immunotherapy is dependent on the metastatic location. Treatment appears more active in lymph nodes compared to other organ sites such as liver, adrenals and bone. Future strategies may include additional local treatment in case of oligoprogression in these organs in patients with otherwise sustained treatment benefit.

PMID: 30171269 [PubMed - as supplied by publisher]

Successful treatment of malignant pheochromocytoma with sacrum metastases: A case report.

Tue, 09/04/2018 - 09:58
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Successful treatment of malignant pheochromocytoma with sacrum metastases: A case report.

Medicine (Baltimore). 2018 Aug;97(35):e12184

Authors: Liu S, Zhou X, Song A, Li WA, Rastogi R, Wang Y, Liu Y

Abstract
RATIONALE: Malignant pheochromocytoma in the spine is a rare disease without standard curative managements so far. The objective of this article is to report a very rare case of malignant pheochromocytoma with sacrum metastases causing severe lumbosacral pain, which was presented with acute radiculopathy and treated with three operations combined with cement augmentation and stabilization. The management of these unique cases has yet to be well-documented.
PATIENT CONCERNS: A 58-year-old man presented with lumbosacral pain, radiating pain and numbness of the right extremity. The patient underwent excision of right adrenal pheochromocytoma in 2010. Imaging studies revealed the density of soft tissues, obvious bony destruction in the sacrum, and significant spinal cord obstruction.
DIAGNOSES: We believe this is the first report of malignant pheochromocytoma with sacrum metastases.
INTERVENTIONS: The patient underwent tissue biopsy and osteoplasty after embolization of the internal iliac artery in January 2015, and exploratory surgery, circumferential spinal cord decompression, and a stabilization procedure via a posterior approach in June 2015 due to spinal canal stenosis caused by cement. Since the position of pedicle screw was not good enough, a revision surgery was performed the next day following the procedures in June 2015.
OUTCOMES: The patient's neurological deficits improved significantly after the third surgery, and the postoperative period was uneventful at the three-year follow-up visit.
LESSONS: We recommend the posterior approach for spinal decompression of the metastatic pheochromocytoma when the tumor has caused neurological deficits. Osteoplasty by cement augmentation is also a good choice for surgical treatment. However, the potential risk of complications in bone cement applications need to be fully recognized.

PMID: 30170467 [PubMed - in process]

Alterations in Sod2-induced oxidative stress affect endocrine cancer progression.

Tue, 09/04/2018 - 09:58
Related Articles

Alterations in Sod2-induced oxidative stress affect endocrine cancer progression.

J Clin Endocrinol Metab. 2018 Aug 24;:

Authors: Ashtekar A, Huk D, Magner A, La Perle KMD, Boucai L, Kirschner LS

Abstract
Context: Although significant advances have been made in understanding the genetics of endocrine tumors, cellular physiology is relatively understudied as a determinant of tumor behavior. Oxidative stress (OS) and reactive oxygen species (ROS) are metabolic factors that may impact tumor behavior, and these are, in part, controlled by MnSod, the mitochondrial superoxide dismutase (encoded by SOD2).
Objective: In this study, we sought to understand the role of the MnSod in the prognosis of aggressive human endocrine cancers. We also aimed to directly assess the effect of MnSod under- or over-expression on tumor behavior using established mouse thyroid cancer models.
Methods: We performed transcriptome analysis of human and mouse models of endocrine cancer. In order to address the role of Sod2 in endocrine tumors, we introduced a Sod2 null allele or a transgenic Sod2 overexpression allele into mouse models of benign thyroid follicular neoplasia or aggressive metastatic follicular thyroid cancer (FTC) and monitored phenotypic changes in tumor initiation and progression.
Results: In the thyroid, SOD2/Sod2 was downregulated in FTC but not papillary thyroid cancer (PTC). Reduced expression of SOD2 was correlated with poorer survival of patients with aggressive thyroid or adrenal cancers. In mice with benign thyroid tumors, Sod2 overexpression increased tumor burden. In contrast, in mice with aggressive FTC, overexpression of Sod2 reduced tumor proliferation and improved mortality, whereas its deficiency enhanced tumor growth.
Conclusions: Overall, our results indicate that SOD2 has dichotomous roles in cancer progression and acts in a context specific manner.

PMID: 30165401 [PubMed - as supplied by publisher]

Mitotane in the treatment of childhood adrenocortical carcinoma: a potent endocrine disruptor.

Tue, 09/04/2018 - 09:58
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Mitotane in the treatment of childhood adrenocortical carcinoma: a potent endocrine disruptor.

Endocrinol Diabetes Metab Case Rep. 2018;2018:

Authors: Oddie PD, Albert BB, Hofman PL, Jefferies C, Laughton S, Carter PJ

Abstract
Adrenocortical carcinoma (ACC) during childhood is a rare malignant tumor that frequently results in glucocorticoid and/or androgen excess. When there are signs of microscopic or macroscopic residual disease, adjuvant therapy is recommended with mitotane, an adrenolytic and cytotoxic drug. In addition to the anticipated side effect of adrenal insufficiency, mitotane is known to cause gynecomastia and hypothyroidism in adults. It has never been reported to cause precocious puberty. A 4-year-old girl presented with a 6-week history of virilization and elevated androgen levels and 1-year advancement in bone age. Imaging revealed a right adrenal mass, which was subsequently surgically excised. Histology revealed ACC with multiple unfavorable features, including high mitotic index, capsular invasion and atypical mitoses. Adjuvant chemotherapy was started with mitotane, cisplatin, etoposide and doxorubicin. She experienced severe gastrointestinal side effects and symptomatic adrenal insufficiency, which occurred despite physiological-dose corticosteroid replacement. She also developed hypothyroidism that responded to treatment with levothyroxine and peripheral precocious puberty (PPP) with progressive breast development and rapidly advancing bone age. Five months after discontinuing mitotane, her adrenal insufficiency persisted and she developed secondary central precocious puberty (CPP). This case demonstrates the diverse endocrine complications associated with mitotane therapy, which contrast with the presentation of ACC itself. It also provides the first evidence that the known estrogenic effect of mitotane can manifest as PPP.
Learning points: Adrenocortical carcinoma is an important differential diagnosis for virilization in young childrenMitotane is a chemotherapeutic agent that is used to treat adrenocortical carcinoma and causes adrenal necrosisMitotane is an endocrine disruptor. In addition to the intended effect of adrenal insufficiency, it can cause hypothyroidism, with gynecomastia also reported in adults.Patients taking mitotane require very high doses of hydrocortisone replacement therapy because mitotane interferes with steroid metabolism. This effect persists after mitotane therapy is completedIn our case, mitotane caused peripheral precocious puberty, possibly through its estrogenic effect.

PMID: 30159150 [PubMed]

Applications of Immunohistochemistry to Endocrine Pathology.

Tue, 09/04/2018 - 09:58
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Applications of Immunohistochemistry to Endocrine Pathology.

Adv Anat Pathol. 2018 Aug 28;:

Authors: Higgins SE, Barletta JA

Abstract
The role of immunohistochemistry (IHC) in endocrine pathology is similar to that in other organ systems in that it can aid in the subclassification of tumors within an organ, confirm site of primary in metastatic disease, provide prognostic information, identify underlying genetic alterations, and predict response to treatment. Although most endocrine tumors do not require IHC to render a diagnosis, there are certain scenarios in which IHC can be extremely helpful. For example, in thyroid, IHC can be used to support tumor dedifferentiation, in the adrenal it can aid in the diagnosis of low-grade adrenocortical carcinomas, and in paragangliomas it can help identify tumors arising as part of an inherited tumor syndrome. This review will focus on the applications of IHC in tumors of the thyroid, parathyroids, adrenals, and paraganglia in adults.

PMID: 30157042 [PubMed - as supplied by publisher]

Catecholamine-Synthesizing Enzymes in Pheochromocytoma and Extraadrenal Paraganglioma.

Tue, 09/04/2018 - 09:58
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Catecholamine-Synthesizing Enzymes in Pheochromocytoma and Extraadrenal Paraganglioma.

Endocr Pathol. 2018 Aug 28;:

Authors: Konosu-Fukaya S, Omata K, Tezuka Y, Ono Y, Aoyama Y, Satoh F, Fujishima F, Sasano H, Nakamura Y

Abstract
In chromaffin cells, tyrosine hydroxylase (TH), aromatic L-amino acid decarboxylase (AADC), dopamine β-hydroxylase (DBH), and phenylethanolamine N-methyltransferase (PNMT) are mainly involved in catecholamine synthesis. In this study, we evaluated the association between the status of catecholamine-synthesizing enzymes and histopathological features of pheochromocytoma and extraadrenal paraganglioma with special emphasis upon their postoperative clinical behavior. Immunohistochemical evaluation of TH, DBH, AADC, PNMT, Ki 67, and S-100 was performed in 29 pheochromocytoma and 10 extraadrenal paraganglioma and one lymph node harboring metastatic pheochromocytoma. Among these cases, metastasis was subsequently developed in three cases. Urinary normetanephrine (U-NM) levels were significantly higher in clinical metastatic cases than non-metastatic ones. Ki 67 labeling index was significantly higher in both clinical metastatic cases and the Adrenal Gland Scaled Score (PASS) score of ≧ 4 cases than PASS < 4 cases, although this score was originally used in pheochromocytoma. H-score of AADC and DBH were significantly lower in PASS ≧ 4 cases than those with < 4 cases, and in the cases associated with intratumoral necrosis (n = 4), the presence of spindle shaped tumor cells (n = 4), and large nests of cells or diffuse growth (n = 5). Lower status of intratumoral AADC could be related to poor differentiation of tumor cells in both catecholamine production and morphology and could be related to aggressive biological behavior of both pheochromocytoma and extraadrenal paraganglioma.

PMID: 30155766 [PubMed - as supplied by publisher]

Perioperative complications of adrenalectomy - 12 years of experience from a single center/teaching hospital and literature review.

Tue, 09/04/2018 - 09:58
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Perioperative complications of adrenalectomy - 12 years of experience from a single center/teaching hospital and literature review.

Arch Med Sci. 2018 Aug;14(5):1010-1019

Authors: Aporowicz M, Domosławski P, Czopnik P, Sutkowski K, Kaliszewski K

Abstract
Introduction: The perioperative complication rate of adrenalectomy varies between 1.7% and 30.7% in the medical literature. This study presents outcomes of adrenalectomy in our center and tries to point out risk factors for perioperative problems.
Material and methods: We retrospectively analyzed all patients who underwent adrenalectomy in our department from January 2004 to June 2015. Patient's sex, indication for procedure, tumor laterality, surgical approach and surgeon's case volume were taken into consideration as possible risk factors for complications.
Results: There were 177 adrenalectomies performed on 170 patients. We reported 18 (10.2%) perioperative complications, 12 (6.8%) surgical and 6 (3.4%) medical. Laparotomy was a significant risk factor for medical (p < 0.01) and overall problems (p = 0.02). Operations more expansive than just adrenalectomy were associated with higher risk of medical complications (p = 0.01). Procedures performed by surgeons with higher volume were associated with smaller risk of medical complications (p < 0.01). Right and left adrenalectomies seem to be related to different kinds of risk - bleeding on the right, injury of surrounding structures on the left (p = 0.05). Patient's sex, indication for procedure, bilateral procedure and side of operation were not statistically significant risk factors for complications.
Conclusions: Adrenal glands are surrounded by various anatomic structures (colon, pancreas, spleen, diaphragm) that may be injured during adrenalectomy. Complications following a laparoscopic procedure may arise from the use of monopolar coagulation and the patient's position on the operating table. High insufflation pressure during retroperitoneoscopic procedures may cause subcutaneous emphysema.

PMID: 30154882 [PubMed]

Educational Case: Pheochromocytoma.

Tue, 09/04/2018 - 09:58
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Educational Case: Pheochromocytoma.

Acad Pathol. 2018 Jan-Dec;5:2374289518780500

Authors: Westover C, Conran RM

Abstract
The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040.

PMID: 30151422 [PubMed]

Adrenal Metastasis of Hepatocellular Carcinoma in Patients following Liver Resection or Liver Transplantation: Experience from a Tertiary Referral Center.

Tue, 09/04/2018 - 09:58
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Adrenal Metastasis of Hepatocellular Carcinoma in Patients following Liver Resection or Liver Transplantation: Experience from a Tertiary Referral Center.

Int J Surg Oncol. 2018;2018:4195076

Authors: Teegen EM, Mogl MT, Pratschke J, Rayes N

Abstract
Introduction: Adrenal metastasis of hepatocellular carcinoma (HCC) is a rare entity and can be treated by resection, local ablative therapy, or systemic therapy. Unfortunately, data about treatment outcome, especially in liver transplant recipients, are rare.
Patients and Methods: From 2005 to 2015, 990 liver resections and 303 liver transplantations because of HCC were performed at our clinic. We retrospectively analyzed treatment outcome of the patients with metachronous adrenal metastasis of HCC, who received either resection, local ablation, or surveillance only.
Results: 10 patients were identified (0.8%). 7 patients received liver transplantation for primary HCC therapy, 3 liver resection, and 1 a local ablative therapy. 8 patients underwent adrenalectomy (one via retroperitoneoscopy), one was treated with local ablation, and one had surveillance only. Seven out of eight patients had no surgical complications and one experienced a pancreatic fistula, treated conservatively. 37.5% of the resected patients had recurrence 1 year after adrenalectomy and 75% after 2 years. The mean survival time after primary diagnosis of HCC was 96.6±22.4 months. After adrenalectomy, the mean survival time was 112.4±25.2 months. The mean time until tumor recurrence was 13.2±3.8 in the total cohort and 15.8±3.8 months in patients after adrenalectomy. The estimated overall survival after adrenalectomy was 77.2±17.4 months.
Conclusion: Metachronous adrenal metastasis occured in less than 1% of HCC patients. Adrenalectomy is a safe procedure and leads to acceptable survival rates even after liver transplantion. Therefore, it should be performed whenever the primary tumor is well controlled and the patient is in adequate physical condition.

PMID: 30151282 [PubMed - in process]

'Malignant' hypertension from hyperaldosteronism: a case report.

Tue, 09/04/2018 - 09:58
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'Malignant' hypertension from hyperaldosteronism: a case report.

Pan Afr Med J. 2018;30:10

Authors: Baradhi KM, Tran T, Mittadodla PS

Abstract
Adrenocortical carcinomas (ACC) are rare with an incidence of 0.7-2 per million population per year and account for only 0.05%-2% of all malignant tumors. While majority of the functional ACC present as Cushing syndrome, recurrent hyperaldosteronism from metastatic ACC is exceedingly rare. We describe a 67-year old female presented with hypertensive urgency & hypokalemia as a result of hyperaldosteronism from an 8-cm right ACC. She underwent a radical right nephrectomy with adrenalectomy that normalized her blood pressure. However, a few years later she presented again with resistant hypertension from hyperaldosteronism, raising the suspicion of recurrence of ACC. A contrast-enhanced CT scan showed a normal left adrenal gland but revealed pulmonary metastases of ACC based on a lung biopsy. Chemotherapy was complicated with side effects leading to refusal of further chemotherapy, henceforth requiring high dose of spironolactone for blood pressure control. Despite curative surgery, metastatic functional ACC should be considered in patients presenting with secondary hypertension from recurrent hyperaldosteronism, due to its high recurrence rate. Besides standard cancer surveillance after a curative surgery, meticulous monitoring of blood pressure is a simple yet crucial way to detect cancer recurrence early.

PMID: 30123413 [PubMed - indexed for MEDLINE]

[Appleby procedure - an option for surgical treatment of pancreatic body tumour infiltrating the celiac axis].

Tue, 09/04/2018 - 09:58
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[Appleby procedure - an option for surgical treatment of pancreatic body tumour infiltrating the celiac axis].

Magy Seb. 2018 03;71(1):16-20

Authors: Kelemen D, Lőcsei Z, Papp R, Ferencz S, Vereczkei A

Abstract
CASE PRESENTATION: After neoadjuvant oncological therapy the surgical treatment of distal pancreatic tumour - infiltrating the celiac axis and the stomach - was reported. During the operation resection of the trunc, distal pancreatectomy, splenectomy, total gastrectomy, resection of the left adrenal gland and cholecystectomy were carried out. The patient's clinical course was uneventful, only transient alteration of liver functions was detected. Histological work-up revealed R1 resection, so adjuvant oncological therapy was decided.
DISCUSSION: Distal pancreatic tumours are frequently inoperable. Infiltration of the celiac axis was similarly considered, however there is a chance for radical operation by the resection of the trunc, when the pancreaticoduodenal arcade will provide the arterial blood supply to the liver. Based on the above case the operative technique and the relevant questions were discussed. In pancreatic tumour and arterial infiltration the preoperative chemotherapy is absolutely recommended, because there is a chance for radical surgery in case of good response.

PMID: 29536751 [PubMed - indexed for MEDLINE]

A case of adrenocortical carcinoma accompanying secondary acute adrenal hypofunction postoperation.

Tue, 09/04/2018 - 09:58
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A case of adrenocortical carcinoma accompanying secondary acute adrenal hypofunction postoperation.

World J Surg Oncol. 2018 Mar 05;16(1):43

Authors: Kou K, Zhang H, Zhang C, Xie E, Chen Y, Wang G, Lv G

Abstract
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare, heterogeneous malignancy with a poor prognosis. ACCs are classified as functioning and non-functioning. The pathogenesis of ACC remains elusive, and diagnosis of ACC is currently based on pathology. In the absence of other effective approaches, surgical resection is the preferred treatment option.
CASE PRESENTATION: Here, we report a case of ACC in the retroperitoneum. The patient underwent radical adrenalectomy and remained disease-free throughout a 6-month follow-up.
CONCLUSIONS: Radical surgical resection is an efficient therapy for ACC, and hydrocortisone can be used to alleviate symptoms of secondary acute adrenal hypofunction.

PMID: 29506536 [PubMed - indexed for MEDLINE]

Managing a Large-Size Adrenal Cyst by Hand-Assisted Laparoscopic Surgery in a Young Male.

Tue, 09/04/2018 - 09:58
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Managing a Large-Size Adrenal Cyst by Hand-Assisted Laparoscopic Surgery in a Young Male.

J Coll Physicians Surg Pak. 2018 Mar;28(3):S28-S30

Authors: Hasan A, Iqbal N, Khawaja MA, Yousuf A, Masood A, Malik Y

Abstract
Adrenal cysts are not common and are most often non-functional and asymptomatic. Most of these cysts are found incidentally. These lesions exhibit a broad histomorphologic spectrum and may vary from benign cysts to malignant cystic neoplasms. Our patient was a 21-year male who presented with abdominal discomfort and epigastric pain and was diagnosed with left adrenal cyst on ultrasound, which was later confirmed by CT scan.

PMID: 29482697 [PubMed - indexed for MEDLINE]

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