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Imaging features of SMARCA4-deficient thoracic sarcomas: a multi-centric study of 21 patients.

Wed, 02/20/2019 - 15:54
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Imaging features of SMARCA4-deficient thoracic sarcomas: a multi-centric study of 21 patients.

Eur Radiol. 2019 Feb 14;:

Authors: Crombé A, Alberti N, Villard N, Pilleul F, Buy X, Le Loarer F, Kind M

Abstract
OBJECTIVES: SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently identified aggressive subtype of sarcoma. The aim of this study was to characterize the CT imaging features of SMARCA4-DTS.
METHODS: From June 2011 to May 2017, 21 adult patients with histologically proven SMARCA4-DTS were identified in the radiological database of 2 French sarcoma reference centers with at least one chest CT scan available. The locations, sizes, heterogeneity, margin definitions, and local extensions of the tumors were reported together with their impact on surrounding organs and regional and distant metastases. Pathological findings, molecular analyses, and patients' outcomes were retrieved.
RESULTS: Of the 21 included patients (median age 48, range 30-74), 18 (85.7%) were male and 18 (85.7%) had a smoking history. Four main radiological patterns were identified depending on the location of the main tumor burden: mediastinal (n = 13), pleural (n = 6), cervical (n = 1), and retroperitoneal (n = 1). Median size was 120 mm (range 46-266). Characteristic CT imaging features of primary tumors included ill-defined margins (n = 21), heterogeneous enhancement after injection (n = 20), multi-compartment extension from mediastinum to lung apex, pleura, or neck (n = 20), compressive effect responsible for atelectasis (n = 11), vascular encasement (n = 16-5 superior vena cava syndrome), and esophagus invasion (n = 5). Primary tumors showed strong 18F-FDG avidity in eight patients with PET-CT. Necrotic lymphadenopathies were found in 19 patients, with a surrounding infiltrate in 13 patients. Metastatic locations at baseline mainly involved adrenal (n = 10), lung (n = 6), and bone (n = 5). Median overall survival was 5 months (range 1-13).
CONCLUSION: Most SMARCA4-DTS present with compressive and infiltrative chest masses with ill-defined necrotic lymphadenopathies. The diagnosis of SMARCA4-DTS should enter in the differentials of the radiologist, especially in the case of a rapidly evolving thoracic mass in young smoking males.
KEY POINTS: • SMARCA4-DTS is a very aggressive poorly differentiated sarcoma with a predilection for young and middle-aged adult male smokers. • SMARCA4-DTS, which is mostly located in the chest cavity, can compress and infiltrate all adjacent organs leading to superior vena syndrome, lung atelectasis, epiduritis, spinal cord compression, and esophagus invasion. • SMARCA4-DTS typically demonstrates several ill-defined necrotic lymphadenopathies spreading in axillar, subclavian, cervical, mediastinum, and retroperitoneum.

PMID: 30762113 [PubMed - as supplied by publisher]

Risk factors for postoperative severe morbidity after pheochromocytoma surgery: A single center retrospective analysis of 262 patients.

Wed, 02/20/2019 - 15:54
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Risk factors for postoperative severe morbidity after pheochromocytoma surgery: A single center retrospective analysis of 262 patients.

Int J Surg. 2018 Dec;60:188-193

Authors: Bai S, Yao Z, Zhu X, Li Z, Jiang Y, Wang R, Wu B

Abstract
PURPOSE: Surgical resection is the primary treatment strategy for pheochromocytoma; however, it carries a high risk of morbidity and mortality. The risk factors for severe morbidity remain unclear and require further exploration. We aimed to identify the risk factors for severe morbidity after pheochromocytoma surgery in Chinese patients.
METHODS: We retrospectively reviewed 262 patients who underwent unilateral laparoscopic or open pheochromocytoma surgery at our center between January 1, 2007 and December 31, 2016. Patient demographics, as well as extensive perioperative data were recorded. Adjusted odds ratios and 95% confidence intervals were determined by multivariate binary logistic regression. Cutoff values and the area under the curve for continuous risk factors were calculated through receiver operating characteristic curve analysis. A P < 0.05 was considered statistically significant.
RESULTS: Of the 262 patients, 78 (29.8%) had severe morbidity. The independent risk factors for severe morbidity were female sex, lower body mass index, coronary heart disease, longer duration of surgery, and intraoperative hemodynamic instability, with odds ratios of 2.624 (P = 0.003), 0.780 (P < 0.001), 2.098, (P = 0.024), 1.005 (P = 0.031), and 2.920 (P = 0.005). The optimal cut off values for body mass index and duration of surgery were 24.25 kg/m2 and 203 min.
CONCLUSIONS: Severe morbidity is common in patients after pheochromocytoma surgery. We identified five independent risk factors for severe morbidity: female sex, lower body mass index, coronary heart disease, longer duration of surgery, and intraoperative hemodynamic instability. Identification of these risk factors may help to improve perioperative strategy.

PMID: 30468902 [PubMed - indexed for MEDLINE]

Changes in the Evaluation and Management of Adrenocortical Carcinoma.

Wed, 02/20/2019 - 15:54
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Changes in the Evaluation and Management of Adrenocortical Carcinoma.

Ann Surg Oncol. 2018 Nov;25(12):3413-3415

Authors: Miller BS

PMID: 30225834 [PubMed - indexed for MEDLINE]

Perioperative serum cortisol levels in ACTH sufficient and ACTH deficient patients during transsphenoidal surgery of pituitary adenoma.

Wed, 02/20/2019 - 15:54
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Perioperative serum cortisol levels in ACTH sufficient and ACTH deficient patients during transsphenoidal surgery of pituitary adenoma.

Endocrine. 2018 10;62(1):83-89

Authors: Borg H, Siesjö P, Kahlon B, Fjalldal S, Erfurth EM

Abstract
PURPOSE: No previous study has analyzed serum cortisol levels during transsphenoidal endoscopic pituitary surgery in patients with and without hydrocortisone (HC) substitution.
METHODS: A total of 15 patients undergoing surgery for a pituitary adenoma were studied. Those with normal ACTH function were either not given HC (n = 7) or received 50 mg intravenous HC at the start of surgery (n = 4). Patients with ACTH deficiency received intravenous HC of 100 mg in the morning before surgery (n = 4) with the additional 50 mg for an afternoon operation (n = 2). Propofol and remifentanil were used as anesthetics. Serum cortisol was measured at the start of and every 30 min during surgery.
RESULTS: Among 7 patients with normal ACTH function without HC substitution, cortisol levels before surgery were 126-244 nmol/L, among the 4 patients undergoing surgery in the morning, whereas the 3 who underwent surgery in the afternoon had lower levels, 38-76 nmol/L. During nose/sinus surgery cortisol levels decreased to 79-139 and 24-54 nmol/L, respectively. At intrasellar manipulation a distinct rise was noted. Also, in the 4 ACTH sufficient patients receiving HC, cortisol levels decreased during nose/sinus surgery, but only with a slight increase during intrasellar surgery. In the 4 ACTH deficient patients cortisol peaked at 1914-2582 nmol/L.
CONCLUSIONS: Patients with normal ACTH function without HC substitution had very low cortisol levels during the first part of surgery, likely suppressed by the anesthetics. After mechanical impact in the sella, a marked increase in cortisol was noted. Supraphysiological cortisol levels were achieved with our routine HC substitution, advising us to reduce the supplementation.

PMID: 29968225 [PubMed - indexed for MEDLINE]

PAX8-Positive B-Cell Lymphoma in Adrenal Gland Masquerading as Metastatic Renal Cell Carcinoma.

Wed, 02/20/2019 - 15:54
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PAX8-Positive B-Cell Lymphoma in Adrenal Gland Masquerading as Metastatic Renal Cell Carcinoma.

Int J Surg Pathol. 2018 Dec;26(8):721-724

Authors: Parakh R, Cheng L, Tretiakova M

Abstract
Extranodal lymphomas involve adrenal glands as a mass lesion in 3% to 5% of cases as identified by imaging studies. Definitive diagnosis of the neoplasm is provided by morphological and immunophenotypical evaluation on limited tissue obtained by fine needle aspiration or core biopsy. We report a diagnostically challenging case that presented as masses involving adrenal gland and lung in the setting of a concurrent primary renal cell carcinoma. PAX8, a frequently used marker for renal cell carcinoma, was immunoreactive in the neoplastic cells of the adrenal mass, and thus highly suggestive for metastatic renal cell carcinoma. The neoplasm was eventually proven to represent PAX8-positive diffuse large B-cell lymphoma after extended immunohistochemical workup. This case of unusual presentation of synchronous renal cell carcinoma and adrenal lymphoma with overlapping PAX8 immunoreactivity serves as a potential diagnostic pitfall with significant clinical implications.

PMID: 29665739 [PubMed - indexed for MEDLINE]

[Therapeutic strategy for the treatment of non-infectious uveitis proposed by an expert panel].

Wed, 02/20/2019 - 15:54
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[Therapeutic strategy for the treatment of non-infectious uveitis proposed by an expert panel].

Rev Med Interne. 2018 Sep;39(9):687-698

Authors: Diwo E, Sève P, Trad S, Bielefeld P, Sène D, Abad S, Brézin A, Quartier P, Koné Paut I, Weber M, Chiquet C, Errera MH, Sellam J, Cacoub P, Kaplanski G, Kodjikian L, Bodaghi B, Saadoun D

Abstract
Conventional immunosuppressive drugs, anti-TNF alpha and other biotherapies used in clinical practice are capable of controlling non-infectious anterior uveitis, posterior uveitis and panuveitis. The present work has been led by a multidisciplinary panel of experts, internists, rheumatologists and ophthalmologists and is based on a review of the literature. In case of corticodependency or sight-threatening disease, conventional immunosuppressive drugs (methotrexate, azathioprine and mycophenolate mofetil) and/or anti-TNF alpha (adalimumab, infliximab) are used to achieve and maintain remission. Interferon is an efficient immunomodulatory treatment, as a second-line therapy, for some therapeutic indications (refractory macular edema, Behçet's vascularitis). Other biologics, especially tocilizumab, are showing promising results. Local treatments (corticosteroids, sirolimus etc.) are adjuvant therapies in case of unilateral inflammatory relapse. Therapeutic response must be evaluated precisely by clinical examination and repeated complementary investigations (laser flare photometry, multimodal imaging, perimetry, electroretinography measures).

PMID: 29610003 [PubMed - indexed for MEDLINE]

The TMEM127 human tumor suppressor is a component of the mTORC1 lysosomal nutrient-sensing complex.

Wed, 02/20/2019 - 15:54
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The TMEM127 human tumor suppressor is a component of the mTORC1 lysosomal nutrient-sensing complex.

Hum Mol Genet. 2018 05 15;27(10):1794-1808

Authors: Deng Y, Qin Y, Srikantan S, Luo A, Cheng ZM, Flores SK, Vogel KS, Wang E, Dahia PLM

Abstract
The TMEM127 tumor suppressor gene encodes a transmembrane protein of unknown function mutated in pheochromocytomas and, rarely, in renal cancers. Tumors with inactivating TMEM127 mutations have increased mTORC1 signaling by undefined mechanisms. Here we report that TMEM127 interacts with the lysosome-anchored complex comprised of Rag GTPases, the LAMTOR pentamer (or 'ragulator') and vATPase, which controls amino acid-mediated mTORC1 activation. We found that under nutrient-rich conditions TMEM127 expression reduces mTORC1 recruitment to Rags. In addition, TMEM127 interacts with LAMTOR in an amino acid-dependent manner and decreases the LAMTOR1-vATPase association, while TMEM127-vATPase binding requires intact lysosomal acidification but is amino acid independent. Conversely, both murine and human cells lacking TMEM127 accumulate LAMTOR proteins in the lysosome. Consistent with these findings, pheochromocytomas with TMEM127 mutations have increased levels of LAMTOR proteins. These results suggest that TMEM127 interactions with ragulator and vATPase at the lysosome contribute to restrain mTORC1 signaling in response to amino acids, thus explaining the increased mTORC1 activation seen in TMEM127-deficient tumors.

PMID: 29547888 [PubMed - indexed for MEDLINE]

Impact of Micro- and Macroscopically Positive Surgical Margins on Survival after Resection of Adrenocortical Carcinoma.

Wed, 02/20/2019 - 15:54
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Impact of Micro- and Macroscopically Positive Surgical Margins on Survival after Resection of Adrenocortical Carcinoma.

Ann Surg Oncol. 2018 May;25(5):1425-1431

Authors: Anderson KL, Adam MA, Thomas SM, Youngwirth L, Stang MT, Scheri RP, Roman SA, Sosa JA

Abstract
PURPOSE: Adrenocortical carcinoma (ACC) is a rare, aggressive cancer; complete surgical resection offers the best chance for long-term survival. The impact of surgical margin status on survival is poorly understood. Our objective was to determine the association of margin status with survival.
METHODS: Patients with ACC were identified from the National Cancer Data Base, 1998-2012, and stratified based on surgical margin status (negative vs. microscopically positive [+] vs. macroscopically [+]). Univariate/multivariate regression/survival analyses were utilized to determine factors associated with margin status and overall survival (OS).
RESULTS: A total of 1553 patients underwent surgery at 589 institutions: 86% had negative, 12% microscopically (+), and 2% macroscopically (+) margins. Those with microscopically (+) and macroscopically (+) margins more often received adjuvant chemotherapy (39.4% macroscopically (+) vs. 38.5% microscopically (+) vs. 25.2% negative margins, p < 0.001). For unadjusted analysis, there was a significant difference in OS between the groups (log-rank p < 0.001), with median survival times of 58 months (95% confidence interval [CI] 49-66) for those with negative margins, 22 months (95% CI 18-34) microscopically (+), and 14 months (95% CI 6-27) macroscopically (+) margins. After adjustment, both microscopically (+) (HR 1.76, p < 0.001) and macroscopically (+) (HR 2.10, p = 0.0019) margin status were associated with compromised survival.
CONCLUSIONS: Having micro- or macroscopically (+) margin status after ACC resection is associated with dose-dependent compromised survival. These results underscore the importance of achieving negative surgical margins for optimizing long-term patient outcomes.

PMID: 29500765 [PubMed - indexed for MEDLINE]

Recontacting Patients with Updated Genetic Testing Recommendations for Medullary Thyroid Carcinoma and Pheochromocytoma or Paraganglioma.

Wed, 02/20/2019 - 15:54
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Recontacting Patients with Updated Genetic Testing Recommendations for Medullary Thyroid Carcinoma and Pheochromocytoma or Paraganglioma.

Ann Surg Oncol. 2018 May;25(5):1395-1402

Authors: Romero Arenas MA, Rich TA, Hyde SM, Busaidy NL, Cote GJ, Hu MI, Gagel RF, Gidley PW, Jimenez C, Kupferman ME, Peterson SK, Sherman SI, Ying A, Bassett RL, Waguespack SG, Perrier ND, Grubbs EG

Abstract
BACKGROUND: No guidelines exist regarding physicians' duty to inform former patients about novel genetic tests that may be medically beneficial. Research on the feasibility and efficacy of disseminating information and patient opinions on this topic is limited.
METHODS: Adult patients treated at our institution from 1950 to 2010 for medullary thyroid cancer, pheochromocytoma, or paraganglioma were included if their history suggested being at-risk for a hereditary syndrome but genetic risk assessment would be incomplete by current standards. A questionnaire assessing behaviors and attitudes was mailed 6 weeks after an information letter describing new genetic tests, benefits, and risks was mailed.
RESULTS: Ninety-seven of 312 (31.1%) eligible patients with an identified mailing address returned the questionnaire. After receiving the letter, 29.2% patients discussed genetic testing with their doctor, 39.3% considered pursuing genetic testing, and 8.5% underwent testing. Nearly all respondents (97%) indicated that physicians should inform patients about new developments that may improve their or their family's health, and 71% thought patients shared this responsibility. Most patients understood the letter (84%) and were pleased it was sent (84%), although 11% found it upsetting.
CONCLUSIONS: Patients believe it is important for physicians to inform them of potentially beneficial developments in genetic testing. However, physician-initiated letters to introduce new information appear inadequate alone in motivating patients to seek additional genetic counseling and testing. Further research is needed regarding optimal methods to notify former patients about new genetic tests and corresponding clinical and ethical implications.

PMID: 29427212 [PubMed - indexed for MEDLINE]

Evidenced-based care of pregnant women with symptomatic adrenal masses - accepting imperfect data.

Wed, 02/20/2019 - 15:54
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Evidenced-based care of pregnant women with symptomatic adrenal masses - accepting imperfect data.

BJOG. 2018 05;125(6):728

Authors: Blumenfeld YJ

PMID: 29090509 [PubMed - indexed for MEDLINE]

[Uveitis in spondyloarthritis].

Wed, 02/20/2019 - 15:54
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[Uveitis in spondyloarthritis].

Z Rheumatol. 2017 Oct;76(8):682-692

Authors: Rudwaleit M, Walscheid K, Heiligenhaus A

Abstract
Acute anterior uveitis (AAU) is the most frequent uveitis subtype. It is often associated with HLA-B27 and with inflammatory rheumatic diseases, in particular with spondyloarthritis (SpA), which itself is strongly associated with HLA-B27. About 40-60% of patients with AAU have an associated spondyloarthritis, and 20-40% of patients with spondyloarthritis also have uveitis. The incidence of AAU in patients with SpA clearly correlates with disease duration. The AAU has an acute onset, usually affects only one eye at a time, and shows a tendency for recurrence. Early therapy of AAU with topical steroids is relevant for good visual outcomes. Minimum duration of therapy of flares of AAU is 6-8 weeks in order to prevent early recurrency. The rate of local complications correlates with the rate of AAU flares and the visual outcome is often good. Refractory uveitis and frequent recurrencies of AAU may be treated with conventional disease-modifying antirheumatic drugs (DMARDs, such as sulfasalazine and methotrexate) and biologicals (e.g. TNF-alpha inhibitors). Any first episode of AAU diagnosed by an ophthalmologist should prompt referral to rheumatology for suspected SpA, particularly if rheumatic symptoms are present.

PMID: 28812149 [PubMed - indexed for MEDLINE]

adrenal tumor; +27 new citations

Wed, 02/13/2019 - 14:40

27 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 2019/02/13

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.

adrenal tumor; +28 new citations

Wed, 02/06/2019 - 13:31

28 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 2019/02/06

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.

adrenal tumor; +38 new citations

Wed, 01/30/2019 - 12:31

38 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 2019/01/30

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.

The Potential of Isoprenoids in Adjuvant Cancer Therapy to Reduce Adverse Effects of Statins.

Wed, 01/23/2019 - 11:26
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The Potential of Isoprenoids in Adjuvant Cancer Therapy to Reduce Adverse Effects of Statins.

Front Pharmacol. 2018;9:1515

Authors: Mo H, Jeter R, Bachmann A, Yount ST, Shen CL, Yeganehjoo H

Abstract
The mevalonate pathway provides sterols for membrane structure and nonsterol intermediates for the post-translational modification and membrane anchorage of growth-related proteins, including the Ras, Rac, and Rho GTPase family. Mevalonate-derived products are also essential for the Hedgehog pathway, steroid hormone signaling, and the nuclear localization of Yes-associated protein and transcriptional co-activator with PDZ-binding motif, all of which playing roles in tumorigenesis and cancer stem cell function. The phosphatidylinositol-4,5-bisphosphate 3-kinase-AKT-mammalian target of rapamycin complex 1 pathway, p53 with gain-of-function mutation, and oncoprotein MYC upregulate the mevalonate pathway, whereas adenosine monophosphate-activated protein kinase and tumor suppressor protein RB are the downregulators. The rate-limiting enzyme, 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR), is under a multivalent regulation. Sterol regulatory element binding protein 2 mediates the sterol-controlled transcriptional downregulation of HMGCR. UbiA prenyltransferase domain-containing protein-1 regulates the ubiquitination and proteasome-mediated degradation of HMGCR, which is accelerated by 24, 25-dihydrolanosterol and the diterpene geranylgeraniol. Statins, competitive inhibitors of HMGCR, deplete cells of mevalonate-derived intermediates and consequently inhibit cell proliferation and induce apoptosis. Clinical application of statins is marred by dose-limiting toxicities and mixed outcomes on cancer risk, survival and mortality, partially resulting from the statin-mediated compensatory upregulation of HMGCR and indiscriminate inhibition of HMGCR in normal and tumor cells. Tumor HMGCR is resistant to the sterol-mediated transcriptional control; consequently, HMGCR is upregulated in cancers derived from adrenal gland, blood and lymph, brain, breast, colon, connective tissue, embryo, esophagus, liver, lung, ovary, pancreas, prostate, skin, and stomach. Nevertheless, tumor HMGCR remains sensitive to isoprenoid-mediated degradation. Isoprenoids including monoterpenes (carvacrol, L-carvone, geraniol, perillyl alcohol), sesquiterpenes (cacalol, farnesol, β-ionone), diterpene (geranylgeranyl acetone), "mixed" isoprenoids (tocotrienols), and their derivatives suppress the growth of tumor cells with little impact on non-malignant cells. In cancer cells derived from breast, colon, liver, mesothelium, prostate, pancreas, and skin, statins and isoprenoids, including tocotrienols, geraniol, limonene, β-ionone and perillyl alcohol, synergistically suppress cell proliferation and associated signaling pathways. A blend of dietary lovastatin and δ-tocotrienol, each at no-effect doses, suppress the growth of implanted murine B16 melanomas in C57BL6 mice. Isoprenoids have potential as adjuvant agents to reduce the toxicities of statins in cancer prevention or therapy.

PMID: 30662405 [PubMed]

Pleural dedifferentiated liposarcoma: A case report.

Wed, 01/23/2019 - 11:26
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Pleural dedifferentiated liposarcoma: A case report.

Mol Clin Oncol. 2019 Jan;10(1):132-136

Authors: Matsukuma S, Oshika Y, Utsumi Y, Obara K, Tanimoto T, Katsurada Y, Takeo H

Abstract
The present case report describes a rare case of pleural liposarcoma. A 45-year-old Japanese man was hospitalized for increasing left chest pain. Imaging revealed a 10-cm pleural tumor and a 1.7-cm contralateral right pulmonary nodule. Biopsy specimens of the pleural tumor showed undifferentiated spindle-shaped and/or rounded sarcomatous features with myxoid stroma. The patient underwent embolization of the arteries feeding the left pleural tumor and palliative partial resection of the pleural tumor. The surgically removed specimens exhibited similar undifferentiated sarcomatous features. The left pleural tumor regrew aggressively, and the patient succumbed to mortality ~4.2 months following hospitalization. Autopsy demonstrated a 35-cm left pleural tumor, metastasizing to both adrenal glands and lumbar vertebral bones, and a 2.2-cm primary adenocarcinoma of the right lung. The majority of the left pleural tumor and its metastases consisted of undifferentiated sarcomatous elements, however, scattered or aggregated lipoblasts were identified in localized areas adjacent to the diaphragm. Immunohistochemically, these lipoblasts were diffusely positive for MDM2 and focally positive for S-100 protein. Undifferentiated sarcomatous tumor cells were focally positive for MDM2 but negative for S-100 protein. This case was diagnosed as pleural dedifferentiated liposarcoma. The local aggressiveness of the pleural liposarcoma directly contributed to the patient's mortality. A review of the literature indicated that the dedifferentiated subtype may serve as a factor that is indicative of a poor prognosis for pleural liposarcoma.

PMID: 30655988 [PubMed]

Aldosterone-producing adrenocortical carcinoma with myxoid differentiation in a cat.

Wed, 01/23/2019 - 11:26
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Aldosterone-producing adrenocortical carcinoma with myxoid differentiation in a cat.

Vet Clin Pathol. 2018 Dec;47(4):660-664

Authors: Attipa C, Beck S, Lipscomb V, English K, Carvalho S, Kiupel M, Szladovits B, Peters LM

Abstract
A 10-year-old male neutered Persian cat was presented with an abdominal mass and history of weakness. Blood smear examination found marked elliptocytosis, and serum biochemical analysis revealed hypokalemia, hypochloremia, increased creatine kinase activity, and a high aldosterone concentration. Cytologic examination of the mass revealed neoplastic endocrine cells with moderate criteria of malignancy, favoring adrenocortical neoplasia. The adrenal mass was surgically excised and histologically characterized by lobules of mildly pleomorphic, polygonal neoplastic cells with moderate to abundant, occasionally granular, eosinophilic cytoplasm. Lobules were separated by fine fibrovascular trabeculae, and numerous cystic cavities containing amorphous eosinophilic material that stained positive with Alcian blue and periodic acid-Schiff were seen. Neoplastic cells were multifocally positive for cytochrome P450 aldosterone synthase. Based on clinicopathologic and immunohistochemical findings the present case was diagnosed as an aldosterone-producing adrenocortical carcinoma with myxoid differentiation. While this entity has not been reported in cats, myxoid differentiation of adrenocortical carcinomas has been found in other species and can pose a major diagnostic challenge on microscopic examination.

PMID: 30240029 [PubMed - indexed for MEDLINE]

[Pheochromocytoma and pregnancy: About one case].

Wed, 01/23/2019 - 11:26
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[Pheochromocytoma and pregnancy: About one case].

Gynecol Obstet Fertil Senol. 2018 09;46(9):667-668

Authors: Valdeyron C, Chartier C, Accoceberry M, Chadeyras JB, Gallot D

PMID: 30120067 [PubMed - indexed for MEDLINE]

Over-diagnosis of potential malignant behavior in MEN 2A-associated pheochromocytomas using the PASS and GAPP algorithms.

Wed, 01/23/2019 - 11:26
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Over-diagnosis of potential malignant behavior in MEN 2A-associated pheochromocytomas using the PASS and GAPP algorithms.

Langenbecks Arch Surg. 2018 Sep;403(6):785-790

Authors: Stenman A, Zedenius J, Juhlin CC

Abstract
PURPOSE: Pheochromocytomas (PCCs) exhibit malignant potential, but current histological modalities for the proper detection of aggressive behavior are debated. The two most widespread algorithms are the "Pheochromocytoma of the Adrenal Gland Scaled Score" (PASS) and the "Grading System for Adrenal Pheochromocytoma and Paraganglioma" (GAPP), both which mostly rely on histological parameters to identify PCC patients at risk of disseminated disease. Since the algorithms are derived from studies using predominantly sporadic PCCs, little is known whether the PASS or GAPP scores can predict malignant potential in hereditary cases.
METHODS: PASS and GAPP were applied on 41 PCCs; 13 PCCs were diagnosed in ten multiple endocrine neoplasia type 2A (MEN 2A) patients carrying established germline RET proto-oncogene mutations, as well as 28 assumed sporadic PCCs.
RESULTS: Six out of thirteen MEN 2A tumors (46%) exhibited PASS scores ≥ 4, indicative of a potential for aggressive behavior. In addition, 7/13 tumors (54%) exhibited GAPP scores ≥ 3, indicative of a "moderately differentiated type" with risk of future recurrence. All MEN 2A PCCs with an elevated PASS score also displayed an elevated GAPP score. In contrast, 4/28 (14%) sporadic PCCs demonstrated PASS scores ≥ 4, and 9/28 (32%) displayed GAPP scores ≥ 3. Follow-up found all cases in the study are free of metastatic or recurrent disease.
CONCLUSIONS: We conclude that the PASS and GAPP scoring systems might be suboptimal for determining true malignant potential in PCCs with constitutional RET mutations and advocate restrictive use of these scores in MEN 2A cases until the results are reproduced in larger numbers.

PMID: 29779047 [PubMed - indexed for MEDLINE]

Adrenocortical Carcinoma with Hypercortisolism.

Wed, 01/23/2019 - 11:26
Related Articles

Adrenocortical Carcinoma with Hypercortisolism.

Endocrinol Metab Clin North Am. 2018 06;47(2):395-407

Authors: Puglisi S, Perotti P, Pia A, Reimondo G, Terzolo M

Abstract
Adrenocortical carcinoma (ACC) is a rare and aggressive tumor. ACC may be associated with different syndromes of hormone excess, most frequently Cushing's syndrome with or without hypersecretion of androgens. Recent data suggest that cortisol excess is a negative prognostic factor in advanced and localized ACC. Surgery with radical intent, when feasible, is the most effective treatment for ACC with hypercortisolism. Mitotane is the medical treatment of choice, both postoperatively and in inoperable or metastatic cases. Because of its slow onset of action, combination with other antisecretory agents (ie, metyrapone) is helpful to achieve more rapid and effective control of hypercortisolism.

PMID: 29754640 [PubMed - indexed for MEDLINE]

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