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NCBI: db=pubmed; Term=adrenal tumor
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Primary adrenal teratoma: A case series and review of the literature.

Tue, 09/18/2018 - 12:31
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Primary adrenal teratoma: A case series and review of the literature.

Mol Clin Oncol. 2018 Oct;9(4):437-442

Authors: Zhou L, Pan X, He T, Lai Y, Li W, Hu Y, Ni L, Yang S, Chen Y, Lai Y

Abstract
Primary adrenal teratoma is a rare type of cancer. Of the 338 patients who underwent adrenalectomy during this study, only 2 (aged 69 and 29 years) were diagnosed with adrenal teratoma and underwent laparoscopic retroperitoneal adrenalectomy. For the purposes of the present study, the term 'adrenal teratoma' was searched in the PubMed database, and 237 articles published between June 1952 and March 2017 were retrieved. However, we were only able to identify 10 relevant studies. In total, these studies reported a series of 18 cases of primary adrenal teratoma in patients aged >16 years, another 8 cases of adult adrenal retroperitoneal teratoma, and 7 cases of adrenal teratoma in children aged <16 years. In the 18 cases aged >16 years, the age range was 17-61 years (mean ± standard deviation, 33.06±15.47 years), the median tumor diameter was 8.25 cm and 13 patients (72.22%) were female. Almost all patients underwent laparoscopic surgery between 2006 and 2017 (75%). Among the 7 cases of adrenal teratoma in children under the age of 16 years, 5 cases (71.43%) were male, the median tumor diameter was 10 cm, the oldest patient was aged 8 years, 5 cases (71.43%) were selected for open surgical resection of the tumor, and 5 cases (71.43%) were followed up without recurrence or death. These data indicate that primary adrenal teratomas in children are rarer compared with adults. Although the data are limited, it was observed that the clinical symptoms of primary adrenal teratoma are not typical, the preferred treatment is retroperitoneal laparoscopic surgery, and the prognosis is favorable. The aim of the present study was to elucidate the clinical characteristics associated with primary adrenal teratoma, in order to further raise awareness of this rare disease.

PMID: 30214733 [PubMed]

AZD8055 inhibits ACTH secretion in a case of bilateral ACTH-secreting pheochromocytoma.

Tue, 09/18/2018 - 12:31
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AZD8055 inhibits ACTH secretion in a case of bilateral ACTH-secreting pheochromocytoma.

Oncol Lett. 2018 Oct;16(4):4561-4566

Authors: Wang F, Tong A, Li C, Cui Y, Sun J, Song A, Li Y

Abstract
Ectopic adrenocorticotropic hormone (ACTH) syndrome is usually caused by pulmonary and bronchial tumors and rarely by pheochromocytoma. To date, the majority of ACTH-secreting pheochromocytomas have been unilateral, with the exception of two cases. A 54-year-old male presented with hypertension and bilateral adrenal tumors. The patient did not report having classic cushingoid features or experience of paroxysmal headaches or sweating, but presented with a slight abdominal obesity. The patient was clinically and pathologically diagnosed with bilateral ectopic ACTH-secreting pheochromocytomas. Whole-exome sequencing demonstrated that the 19 pheochromocytoma-related genes were unmutated. The pheochromocytomas on the two sides exhibited negative ACTH staining, but the ACTH concentration was markedly higher in the tumor tissue homogenates than in those tumors of another 3 patients with non-ACTH secretion pheochromocytoma. Electron microscopy identified two types of neuroendocrine cells in the tumor tissues. Primary culture of the pheochromocytoma cells revealed that ACTH secretion was inhibited by a mechanistic target of rapamycin inhibitor, AZD8055.

PMID: 30214591 [PubMed]

Anaplastic Kaposi's Sarcoma of the Adrenal in an HIV-negative Patient With Literature Review.

Tue, 09/18/2018 - 12:31
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Anaplastic Kaposi's Sarcoma of the Adrenal in an HIV-negative Patient With Literature Review.

Adv Anat Pathol. 2018 Sep 12;:

Authors: Bisceglia M, Minenna E, Altobella A, Sanguedolce F, Panniello G, Bisceglia S, Ben-Dor DJ

Abstract
Kaposi's sarcoma (KS) is a peculiar tumor of viral etiology, with the HHV8 rhadinovirus playing a fundamental role in its development. Several epidemiological categories of KS have been identified, of which the sporadic, endemic, iatrogenic, and the epidemic are the main ones. Several histologic disease morphologies have been described, such as inflammatory, angiomatous, spindle cell, mixed, and the anaplastic (sarcomatous) subtypes. The skin of the limbs is most commonly affected, but any other organ or site may be involved. Microscopically KS may enter the differential diagnosis with several different entities, and for this purpose the immunohistochemical detection of the viral latent nuclear antigen-1 (LNA-1) may be crucial. Sporadic KS is usually benign, but rarely it may be aggressive. Anaplastic histology heralds an ominous course in any clinical context. We report a case of anaplastic retroperitoneal KS, occurring in an HIV-negative adult man. This patient presented with a huge left suprarenal mass, which was totally resected, and initially diagnosed as inflammatory leiomyosarcoma, because of the monomorphic spindle cell tumor morphology. After 12 years the tumor recurred locally as an unresectable mass, which was biopsied and examined. At the time of recurrence, the histologic slides of the primary tumor were reviewed, and the previous diagnosis was changed to that of atypical KS. Histologically the recurrent tumor showed both spindle cell and epithelioid appearances. Strongly diffuse HHV8/LAN-1 immunopositivity was documented in both tumors. The final diagnosis for the entire case was anaplastic KS. Then, the patient died in a few months.

PMID: 30212382 [PubMed - as supplied by publisher]

Large adrenal cyst masquerading as a pancreatic cystic tumor: a rare diagnosis based on endoscopic ultrasound.

Tue, 09/18/2018 - 12:31
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Large adrenal cyst masquerading as a pancreatic cystic tumor: a rare diagnosis based on endoscopic ultrasound.

Endosc Int Open. 2018 Sep;6(9):E1109-E1111

Authors: Voudoukis E, Velegraki M, Khamaysi I, Karoumpalis I, Kazamias G, Fragaki M, Mastorakis E, Paspatis G

Abstract
Adrenal cystic lesions constitute a rare finding and usually are diagnosed incidentally as retroperitoneal cysts during imaging studies. A major issue, especially for the large-sized ones, is their preoperative diagnosis and management, as imaging modalities often fail to detect their exact origin prior to surgery. We report a case of a big adrenal cyst that was mistakenly diagnosed and followed up as pancreatic cystic tail tumor. Our thorough workup, which included endoscopic ultrasound (EUS), managed to delineate an adrenal gland-originated cyst and altered further management of the patient. According to our knowledge, this is the first report in literature in which EUS proved its superiority over other imaging studies in clarifying the origin of an ambiguous big retroperitoneal cyst. We believe that for cases of large retroperitoneal cystic lesions, EUS consists the optimal diagnostic and sampling tool.

PMID: 30211299 [PubMed]

A Case of Bilateral Adrenocortical Adenomas along with Virilization and Cushing's Syndrome.

Tue, 09/18/2018 - 12:31
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A Case of Bilateral Adrenocortical Adenomas along with Virilization and Cushing's Syndrome.

Intern Med. 2018 Sep 12;:

Authors: Kobayashi T, Imachi H, Sato S, Ibata T, Fukunaga K, Yoshimoto T, Kikuchi F, Yonezaki K, Yamaji N, Lyu J, Dong T, Nagata H, Kadota K, Kushida Y, Haba R, Murao K

Abstract
We herein present the case of a 27-year-old woman with clinical and biochemical features of virilism. Imaging studies revealed the presence of a bilateral adrenal tumor. Although the secretion of androgens was remarkable, the autonomous production of cortisol was also evident because of a loss of circadian rhythm and the absence of cortisol suppression by dexamethasone. The surgical excision of both adrenal tumors was performed, and the histological examination showed no malignancy. We also report the successful pregnancy and delivery of the patient who showed evolving adrenocortical insufficiency along with virilization and Cushing' s syndrome and who continued to receive glucocorticoid replacement therapy during pregnancy.

PMID: 30210105 [PubMed - as supplied by publisher]

Can Adrenal Adenomas Be Differentiated From Adrenal Metastases at Single-Phase Contrast-Enhanced CT?

Tue, 09/18/2018 - 12:31
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Can Adrenal Adenomas Be Differentiated From Adrenal Metastases at Single-Phase Contrast-Enhanced CT?

AJR Am J Roentgenol. 2018 Sep 12;:1-7

Authors: Tu W, Verma R, Krishna S, McInnes MDF, Flood TA, Schieda N

Abstract
OBJECTIVE: The purpose of this study is to evaluate whether adrenal metastases can be reliably differentiated from adenomas at single-phase contrast-enhanced CT.
MATERIALS AND METHODS: Sixty-one consecutive patients from a single-institution lung cancer registry (40 metastases and 36 adenomas) who underwent single-phase contrast-enhanced CT at baseline diagnosis were retrospectively studied by two radiologists (blinded to the diagnoses) who independently evaluated four features previously described in adenomas: smooth margin, rim enhancement, central vein sign (preserved adrenal vein), and homogeneity (using a 5-point Likert scale). A third radiologist measured size and attenuation and performed quantitative texture analysis. Comparisons were performed using chi-square, logistic regression, and ROC analysis.
RESULTS: Metastases were larger than adenomas (mean [± SD] 24 ± 11 mm [range, 11-66 mm] vs 19 ± 5 mm [range, 11-34 mm]; p = 0.012), with overlap between groups. Attenuation of metastases and adenomas did not differ significantly (58.2 ± 21.0 HU [range, 21.0-108.0] vs 55.5 ± 21.5 HU [range, 14.0-105.0]; p = 0.582). Skewness and kurtosis did not differ between groups (p = 0.612 and 0.978, respectively), whereas entropy was higher in metastases (p = 0.013). The AUC for entropy to diagnose metastases was 0.65 (95% CI, 0.52-0.77). Tumor margin, rim enhancement, and the central vein sign did not differ between groups (p > 0.05). Metastases were considered more heterogeneous by both radiologists (p = 0.001 and 0.011, respectively), and agreement was satisfactory (κ = 0.51). Likert scores of 4 or 5 (mostly or completely heterogeneous) yielded sensitivity and specificity for diagnosis of metastases of 32.5% and 97.2%, respectively, for radiologist 1 and 22.5% and 97.2%, respectively, for radiologist 2.
CONCLUSION: Adrenal metastases cannot be reliably differentiated from adenomas at single-phase contrast-enhanced CT. Increased tumor size and heterogeneity were specific findings but showed unacceptably low sensitivity.

PMID: 30207794 [PubMed - as supplied by publisher]

Vagus Nerve Stimulation at the Interface of Brain-Gut Interactions.

Tue, 09/18/2018 - 12:31
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Vagus Nerve Stimulation at the Interface of Brain-Gut Interactions.

Cold Spring Harb Perspect Med. 2018 Sep 10;:

Authors: Bonaz B, Sinniger V, Pellissier S

Abstract
The vagus nerve, a key component of the cross-communication between the gut and the brain, is a major element of homeostasis sensing the "milieu intérieur" and boosting the nervous and endocrine responses to maintain the gastrointestinal health status. This nerve has anti-inflammatory properties regulating the gut through the activation of the hypothalamic-pituitary-adrenal axis and the release of cortisol and through a vagovagal reflex, which has an anti-tumor necrosis factor (TNF) effect called the cholinergic anti-inflammatory pathway. Stimulating this nerve is an interesting tool as a nondrug therapy for the treatment of gastrointestinal diseases in which brain-gut communication is dysfunctional, such as inflammatory bowel disorders and others. This review presents the rationale of vagal gastrointestinal physiology and diseases and the most recent advances in vagus nerve stimulation. It also highlights the main issues to be addressed in the future to improve this bioelectronic therapy for gastrointestinal disorders.

PMID: 30201788 [PubMed - as supplied by publisher]

Cardiac pheochromocytoma encasing the left main coronary artery.

Tue, 09/18/2018 - 12:31
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Cardiac pheochromocytoma encasing the left main coronary artery.

J Card Surg. 2018 Apr;33(4):176-178

Authors: Alraddadi HO, Alsagheir A, Belley-Côté EP, McClure GR, Whitlock RP

PMID: 29573472 [PubMed - indexed for MEDLINE]

Recurrent epistaxis leading to diagnosis of primary sinonasal melanoma.

Tue, 09/18/2018 - 12:31
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Recurrent epistaxis leading to diagnosis of primary sinonasal melanoma.

BMJ Case Rep. 2018 Feb 16;2018:

Authors: Tawagi K, Akaraz-Avedissian R, Burman-Solovyeva I

Abstract
A 48-year-old man presented to urgent care with recurrent epistaxis over 6 months. Initially, nosebleeds were controlled with packing or cautery. Ultimately, he was referred to ear, nose and throat department and underwent nasal endoscopy which revealed polypoid tissue. A biopsy of the polyp showed non-specific inflammation with no evidence of malignancy. Follow-up maxillofacial CT revealed a large mass lesion in the right maxillary sinus, right nasal fossa, much of the ethmoids and right sphenoid, with destruction of adjacent bony structures. MRI revealed a mass in the right nasal cavity with extension into the ethmoid and anterior sphenoid sinus, anterior cranial fossa and medial orbits. Staging CT discovered metastatic disease in the adrenal glands and lymphadenopathy in the neck. The patient underwent endoscopic sinus surgery with debulking and tissue diagnosis of malignant melanoma. He completed radiation therapy to sinus and was subsequently enrolled in a clinical trial. Most recent imaging revealed complete metabolic response on positron emission tomography.

PMID: 29453209 [PubMed - indexed for MEDLINE]

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
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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]

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