Adrenocortical Carcinoma With Concomitant Myelolipoma in a Patient With Hyperaldosteronism

2005 ◽  
Vol 129 (6) ◽  
pp. e144-e147 ◽  
Author(s):  
Xichun Sun ◽  
Alberto Ayala ◽  
Claudia Y. Castro
Keyword(s):  
Adrenocortical Carcinoma ◽  
Severe Hypertension ◽  
Serum Potassium Level ◽  
Venous Invasion ◽  
Ki 67 ◽  
Unilateral Adrenalectomy ◽  
Aldosterone Level ◽  
Serum Aldosterone ◽  
Mitotic Figures ◽  
Atypical Mitosis

Abstract We present a case of aldosterone-secreting adrenocortical carcinoma with concomitant myelolipoma. To the best of our knowledge, this is the first such reported case. The patient was a 43-year-old man with severe hypertension. Clinical workup revealed an increased serum aldosterone level, hypokalemia, and metabolic alkalosis, and a left adrenal mass was found on computed tomography. The patient underwent a unilateral adrenalectomy, which led to improvement in blood pressure, the serum potassium level, and aldosterone concentration. The tumor weighed 70 g and measured 5.0 cm. On microscopic examination, we found necrosis, focal cytologic atypia, diffuse eosinophilic cells comprising more than 75% of the tumor, 5 to 7 mitotic figures per 50 high-power fields, rare atypical mitosis, and venous invasion. At the periphery of the tumor but within the capsule, microscopic areas of myelolipoma were seen. Ki-67 staining was positive in 20% of the tumor cells. Although rare, aldosterone-secreting carcinoma associated with myelolipoma should be included in the differential diagnosis of adrenal gland masses.

scholarly journals Case Report: Ectopic Adrenocortical Carcinoma in the Ovary

2021 ◽  
Vol 12 ◽  
Author(s):  
Wen-Hsuan Tsai ◽  
Tze-Chien Chen ◽  
Shuen-Han Dai ◽  
Yi-Hong Zeng
Keyword(s):  
Adrenocortical Carcinoma ◽  
Performance Status ◽  
Poor Performance ◽  
Abdominal Circumference ◽  
Poor Performance Status ◽  
Test Results ◽  
Ki 67 ◽  
Complete Survey ◽  
Rare Malignancy ◽  
Captopril Test

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. ACCs are rare and usually endocrinologically functional. We present the case of a 59-year-old woman who experienced abdominal fullness for 6 months and increased abdominal circumference. A large pelvic tumor was observed. She underwent cytoreductive surgery and the pathological test results revealed local tumor necrosis and prominent lympho-vascular invasion. Neuroendocrine carcinoma was the first impression, but positivity for synaptophysin, alpha-inhibin, transcription factor enhancer 3 (TFE-3), calretinin (focal), and CD56 (focal) and high Ki-67-labeling proliferating index (>80%) confirmed the diagnosis of ectopic ACC. Ectopic primary aldosteronism could not be excluded. However, we did not perform saline infusion test or captopril test due to poor performance status. When pathological test reports reveal neuroendocrine features not typically found in the organ being examined, IHC staining should be performed to rule out ectopic ACC. Whether the ectopic ACC is functional or not requires complete survey.

scholarly journals Primary epithelioid rhabdomyosarcoma of the stomach: a case report and review of literature

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Yangkun Wang ◽  
Pei Guo ◽  
Zhishang Zhang ◽  
Runde Jiang ◽  
Zuguo Li
Keyword(s):  
Tumor Cells ◽  
Unusual Case ◽  
Histopathological Examination ◽  
Gastroesophageal Junction ◽  
Rare Tumor ◽  
Review Of Literature ◽  
Ki 67 ◽  
Case Presentation ◽  
Cytoplasmic Content ◽  
Mitotic Figures

Abstract Background Epithelioid rhabdomyosarcoma is a rare tumor that generally occurs in the bladder, the parotid gland, or the skin of the neck. We describe an unusual case of primary epithelioid rhabdomyosarcoma of the stomach and review the literature. Case presentation A 64-year-old woman presented with a lesion at the gastroesophageal junction. Histopathological examination showed irregularly sized round cells with low cytoplasmic content and eccentric nuclei. Mitotic figures were present. Fibrovascular septa and areas of necrosis were observed between tumor cells. Tumor cells were strongly positive for MyoD1, desmin, and myogenin, and weakly positive for actin, CD56, and PGP9.5. The ki-67 index was ≥90%. Conclusions Primary epithelioid rhabdomyosarcoma of the stomach is extremely rare. Better awareness of this entity is necessary for early diagnosis and treatment.

scholarly journals CCNB2 and AURKA overexpression may cause atypical mitosis in Japanese cortisol-producing adrenocortical carcinoma with TP53 somatic variant

PLoS ONE ◽  
2020 ◽  
Vol 15 (4) ◽  
pp. e0231665
Author(s):  
Akira Ikeya ◽  
Mitsuko Nakashima ◽  
Miho Yamashita ◽  
Keisuke Kakizawa ◽  
Yuta Okawa ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Somatic Variant ◽  
Atypical Mitosis

scholarly journals Adrenocortical Carcinoma With Renal Vein Thrombus Extended to Inferior Vena Cava: A Case Report

2015 ◽  
Vol 100 (7-8) ◽  
pp. 1190-1193 ◽  
Author(s):  
Mohsen Ayati ◽  
Jafar Shahbazi ◽  
Ali Tehranchi ◽  
Elnaz Ayati ◽  
Yousef Rezaei
Keyword(s):  
Inferior Vena Cava ◽  
Adrenocortical Carcinoma ◽  
Renal Vein ◽  
Inferior Vena ◽  
Vena Cava ◽  
Venous Invasion ◽  
Survival Duration ◽  
Comprehensive Therapy ◽  
Advanced Stages ◽  
Rare Malignancy

Adrenocortical carcinoma (ACC) is a rare aggressive tumor. Renal vein and inferior vena cava (IVC) thrombi have been found as uncommon presentations of ACC; however, the implementation of comprehensive therapy has remained controversial in such cases. We report a case of a 46-year-old woman with a large ACC associated with the invasion of tumor to IVC confirmed by imaging and immunohistochemistry examinations. The patient was treated successfully using aggressive surgery, including adrenalectomy and thrombectomy adjunct to an adrenocorticolytic agent. However, she died of metastasis complications at 3-month follow-up period. ACC is a rare malignancy, mostly presenting in advanced stages with poor prognosis. Implementing aggressive surgical therapy might be effective for the management of such cases; however, the short survival duration in our case underscores the need for defining the precise therapy of metastatic ACC associated with venous invasion.

scholarly journals Results of the ADIUVO Study, the First Randomized Trial on Adjuvant Mitotane in Adrenocortical Carcinoma Patients

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A166-A167
Author(s):  
Massimo Terzolo ◽  
Martin Fassnacht ◽  
Paola Perotti ◽  
Rossella Libe ◽  
André Lacroix ◽  
...  
Keyword(s):  
Observational Study ◽  
Adrenocortical Carcinoma ◽  
Multivariable Analysis ◽  
Stage I ◽  
Controlled Study ◽  
Intermediate Risk ◽  
Ki 67 ◽  
Risk Of Recurrence ◽  
Randomized Controlled ◽  
Baseline Characteristics

Abstract Background: The ESE-ENSAT guidelines on the management of adrenocortical carcinoma (ACC) suggest adjuvant mitotane for patients at high risk of recurrence following radical surgery. This indication has a limited evidence base, lacking results from randomized controlled trials. No suggestion for or against adjuvant mitotane in low-risk patients was given, since studies did not stratify patients for prognosis. The randomized controlled study ADIUVO compared the efficacy of adjuvant mitotane treatment vs. observation in prolonging recurrence-free survival (RFS) in ACC patients at low-intermediate risk of recurrence. Methods: The main inclusion criteria were: stage I-III ACC, R0 surgery, and Ki-67 ≤10%. Patients were randomly assigned 1:1 to adjuvant mitotane (MIT) or observation (OBS). The primary endpoint of the study was RFS. Patients who refused randomization were offered inclusion in the ADIUVO OBSERVATIONAL study. In this prospective, observational study, patients were managed as in the ADIUVO study. A total of 91 patients were enrolled in ADIUVO, 45 in the MIT and 46 in the OBS arm. Baseline characteristics of patients were perfectly matched between the 2 arms: median age, 51 vs. 50.5 years; female, 73% vs. 67%; stage I, 20% vs. 26%; stage II, 67% vs. 63%, stage III, 13% vs. 11%; ACC secretion 44% vs. 36%; Weiss 5 vs. 5; respectively. In ADIUVO OBSERVATIONAL, 42 patients were treated with mitotane and 53 were untreated. Baseline characteristics of patients were matched between the 2 groups and with MIT and OBS groups in ADIUVO. Thus, the ADIUVO OBSERVATIONAL cohorts could be analyzed in parallel to those of ADIUVO. Results: In the ADIUVO study, recurrences were 8 in the MIT and 11 in the OBS arm, while deaths were 2 and 5, respectively. RFS and overall survival (OS) did not significantly differ between the 2 arms. Tumor size was a predictor of RFS in multivariable analysis. In the OBS arm, the HR for recurrence was 1.321 (95%CI, 0.55–3.32, p=0.54) and HR for death 2.171 (95%CI, 0.52–12.12, p=0.29). The survival analysis in the ADIUVO OBSERVATIONAL study confirmed the findings of ADIUVO. Given the outcome of both studies, the NNT is 55. Conclusions: ACC patients at low-intermediate risk of recurrence after surgery are a minority; however, they show a far better prognosis than expected (5-year RFS is about 75%) and do not benefit significantly from adjuvant mitotane. The results of the ADIUVO study do not support routine use of adjuvant mitotane in this subset of patients, who may thus avoid a potentially toxic treatment. This is an important step toward personalization of ACC care.

scholarly journals SUN-165 Acnes Fulminans in a Very Young Patient with Adrenal Carcinoma After Surgical Treatment

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ricardo Kunde Minuzzi ◽  
Fabiana Jaeger ◽  
Bruna Tomasi Lorentz ◽  
Maria Eduarda Bilhar Cruxen ◽  
Petry Carolina ◽  
...  
Keyword(s):  
Young Patient ◽  
Corticosteroid Therapy ◽  
Adrenocortical Carcinoma ◽  
Serum Cortisol ◽  
Normal Serum ◽  
Total Testosterone ◽  
Ki 67 ◽  
Severe Acne ◽  
Acne Fulminans

Abstract Introduction: Acne fulminans is a rare disease, which most often affects teenagers and young adults, being described associated with exogenous testosterone use and with elevated adrenal androgens in congenital adrenal hyperplasia. We describe a case of acne fulminans in a toddler with adrenocortical carcinoma. Clinical Case: A 2.3 years-old male diagnosed with adrenocortical tumor presenting pubarche, increased penis and body hair, severe acne lesions in face, chest and back and also arterial hypertension. He was previously healthy, with normal development at his first year. The investigation showed adrenal hyperandrogenism and hypercortisolism. Serum values of Total Testosterone >1500 ng/dL (<7,00 - 25,91 ng/dL), ACTH: 7,2 pg/mL (<46 pg/mL), DHEAS: 682 µg/dL (<15 µg/dL), Aldosterone: 11 ng / dL (2.5-39.2 ng /dL), Serum Cortisol 8AM: 30.65µg/dL (5.27 to 22.45 µg/dL), Salivary midnight cortisol: 29.5nmol/L (11:30PM-00:30AM < 7.6 nmol/L). The abdominal computed tomography (CT) showed the left suprarenal gland with a 5,1 x 3,9 cm lesion. Left adrenalectomy was performed, whose histology confirmed adrenocortical carcinoma, without exceeding the capsule, but with a vascular invasion focus and Ki-67 rate of 20%. Hormonal levels in the early follow up were normal and prophylactic corticosteroid therapy was progressively removed. At the three months after surgery outpatient follow-up, worsening of his skin acneiform lesions was observed. He presented painful papules, pustules and crusts at face, chest and back, with purulent exudation and bleeding lesions, associated with axillary and inguinal adenomegaly and acne fulminans was diagnosed. Clinical and ultrasonographic examination also showed the presence of hepatosplenomegaly,. The treatment was initially made with oral corticosteroids and antibiotics. At this time, laboratory tests of androgens, DHEAS and salivary cortisol were normal, but inflammatory markers were high. A new postoperative abdominal CT showed, at the left adrenal topography, two lesions, 47HU attenuation, 1.5 x 1.2 x 1.1cm and 2.3 x 1.7 x 2.0cm, and also confirmed mild hepatosplenomegaly. Biopsy of inguinal lymph nodes was performed, and histology showed lymphoid follicles hyperplasia. Conclusions: This case report showing the association between acne fulminans and adrenocortical carcinoma, at postoperative time, when the child already had normal serum androgens and after hypercortisolism resolution, without oral corticosteroid therapy. Disordered immune response and/or hypersensitivity reaction to Propionibacterium acnes antigens, are also considered as possible triggering factors, although the etiology for this cases is not well established. The description of an acne fulminans case in such a young patient with adrenocortical carcinoma seems unusual and such association deserves further elucidation.

scholarly journals A Silent Corticotroph Pituitary Carcinoma: Lessons From an Exceptional Case Report

2021 ◽  
Vol 12 ◽  
Author(s):  
Pablo Remón-Ruiz ◽  
Eva Venegas-Moreno ◽  
Elena Dios-Fuentes ◽  
Juan Manuel Canelo Moreno ◽  
Ignacio Fernandez Peña ◽  
...  
Keyword(s):  
Case Report ◽  
Primary Tumor ◽  
Pituitary Tumor ◽  
Pituitary Tumors ◽  
Treatment Resistance ◽  
Exceptional Case ◽  
Ki 67 ◽  
Fractionated Radiotherapy ◽  
Mitotic Figures

Nowadays, neither imaging nor pathology evaluation can accurately predict the aggressiveness or treatment resistance of pituitary tumors at diagnosis. However, histological examination can provide useful information that might alert clinicians about the nature of pituitary tumors. Here, we describe our experience with a silent corticothoph tumor with unusual pathology, aggressive local invasion and metastatic dissemination during follow-up. We present a 61-year-old man with third cranial nerve palsy at presentation due to invasive pituitary tumor. Subtotal surgical approach was performed with a diagnosis of silent corticotroph tumor but with unusual histological features (nuclear atypia, frequent multinucleation and mitotic figures, and Ki-67 labeling index up to 70%). After a rapid regrowth, a second surgical intervention achieved successful debulking. Temozolomide treatment followed by stereotactic fractionated radiotherapy associated with temozolomide successfully managed the primary tumor. However, sacral metastasis showed up 6 months after radiotherapy treatment. Due to aggressive distant behavior, a carboplatine-etoposide scheme was decided but the patient died of urinary sepsis 31 months after the first symptoms. Our case report shows how the presentation of a pituitary tumor with aggressive features should raise a suspicion of malignancy and the need of follow up by multidisciplinary team with experience in its management. Metastases may occur even if the primary tumor is well controlled.

scholarly journals Description of a Novel Murine Model for Ileocystoplasty and Early Histologic Changes

2011 ◽  
Vol 11 ◽  
pp. 1325-1331 ◽  
Author(s):  
Matthew R. Braasch ◽  
Thomas S. Griffith ◽  
Christopher S. Cooper ◽  
J. Christopher Austin
Keyword(s):  
Murine Model ◽  
Cellular Proliferation ◽  
Survival Rates ◽  
Bladder Augmentation ◽  
Ki 67 ◽  
Show Evidence ◽  
Cell Layers ◽  
Similar Weight ◽  
Mitotic Figures ◽  
Histologic Changes

There is concern that bladder augmentation with bowel segments predisposes toward the development of carcinoma. Additionally, patients with neurogenic bladder and bladder cancer often present with advanced stage and have poor survival. Cellular hyperproliferation at the urointestinal junction (UIJ) has been implicated in this scenario. We aimed to develop a reproducible murine model of ileocystoplasty (ICP). We also performed preliminary analysis of any early histologic changes with focus on cellular proliferation at the UIJ. Fifteen 6- to 8-week-old female C57BL/6 mice underwent ICP, where a 1-cm ileal segment was used for bladder augmentation. Four sham mice underwent cystotomy and closure, and four mice did not undergo surgery. The mice were euthanized at 12 weeks postsurgery, and paraffin sections were stained for hematoxylin and eosin (H&E). Cellular proliferation was investigated using Ki-67. A novel model of ICP in mice was developed and demonstrated to be technically feasible in approximately 60 min under the operating microscope. Twelve-week postsurgical survival rates were 80% (12 of 15). The surviving mice had a similar weight gain as the sham mice. H&E sections showed thickened urothelium (six to 10 cell layers) at the UIJ, but sparse mitotic figures and no dysplastic changes. Ki-67 staining was rare in the urothelium, and showed no differences between the sham and ICP mice in the bladder or at the UIJ. We here demonstrate the first murine model of ICP. Preliminary studies did not show evidence of early hyperproliferation at the UIJ or in the bladder, but further long-term studies as well as studies with transgenic mice are warranted.

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