Mixed corticomedullary adrenal carcinoma – case report: Comparison in features, treatment and prognosis with the other two reported cases

Alsabek, Mhd Belal; Alhmaidi, Riad; Ghazzawi, Bader Y. Al; Hamed, Ghiath; Alseoudi, Alhadi

doi:10.1016/j.ijscr.2017.01.010

Cited by 9 publications

(23 citation statements)

References 17 publications

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“…Mixed corticomedullary tumors are extremely rare, including the present one, and there are only 26 cases reported in the English literature since its initial description in 1969 by Mathison et al [16]. However, the medullary component has usually been reported as a benign pheochromocytoma [17], and the adrenocortical component can be either an adenoma [1-3, 6, 7, 9] or a carcinoma [4,5,8]. ere have been only 3 previously reported cases of malignant, mixed adrenocortical tumors [4,5,8].…”

Section: Discussionmentioning

confidence: 92%

“…However, the medullary component has usually been reported as a benign pheochromocytoma [17], and the adrenocortical component can be either an adenoma [1-3, 6, 7, 9] or a carcinoma [4,5,8]. ere have been only 3 previously reported cases of malignant, mixed adrenocortical tumors [4,5,8]. In one of these cases [4], the medullary and cortical tum or cells appeared to collide with each other, whereas in the other two patients [5,8], like in our case, the two cell populations do not collide but are intermingled instead.…”

Section: Discussionmentioning

confidence: 99%

“…Mixed corticomedullary adrenal tumors (MCATs) are very rare and present as a single heterogeneous mass composed of intimately admixed adrenocortical and medullary cells [1][2][3][4][5][6][7][8][9]. e medullary component usually consists of pheochromocytes, whereas the cortical component is usually an adrenocortical adenoma or less frequently an adrenocortical carcinoma [1][2][3][4][5][6][7][8][9]. Clinical and/or biochemical evidence of cortisol hypersecretion, putatively by the cortical component, has been documented in about half of the reported cases of mixed corticomedullary adrenal tumors [1][2][3][4][5][6][7][8][9].…”

Section: Introductionmentioning

confidence: 99%

“…e medullary component usually consists of pheochromocytes, whereas the cortical component is usually an adrenocortical adenoma or less frequently an adrenocortical carcinoma [1][2][3][4][5][6][7][8][9]. Clinical and/or biochemical evidence of cortisol hypersecretion, putatively by the cortical component, has been documented in about half of the reported cases of mixed corticomedullary adrenal tumors [1][2][3][4][5][6][7][8][9]. ese tumors are especially rare in young patients.…”

Section: Introductionmentioning

confidence: 99%

“…ese tumors are especially rare in young patients. On the contrary, catecholamine hypersecretion by the pheochromocytoma component appears to be less frequent [1][2][3][4][5][6][7][8][9]. A significant proportion of MCAT is clinically and biochemically nonfunctioning lesions.…”

Section: Introductionmentioning

confidence: 99%

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From ACTH-Dependent to ACTH-Independent Cushing’s Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

Ramírez‐Rentería

Espinosa-de-los-Monteros

Espinosa-Cárdenas

et al. 2020

Case Reports in Endocrinology

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Context. Truly, mixed corticomedullary adrenal tumors (MCATs) may not come from colliding tumors but from a single stem cell. Objective. To report the immunohistochemical and molecular evaluation of a patient with ectopic ACTH syndrome (EAS) from a MCAT which has single cells with features of both 96 medullary and cortical differentiation. Case Description and Methods. A 16-year-old woman presented with severe EAS and a large right MCAT composed of ACTH-secreting cells resembling pheochromocytoma and another lineage similar to adrenal carcinoma. Immunohistochemistry (IHC) showed positivity for medullary (ACTH, chromogranin A, synaptophysin, and PS-100) and epithelial components (inhibin, melan-A, and calretinin). Embryonic stem cell markers were evaluated using RT/PCR and immunofluorescence. After initial surgery, the tumor recurred shifting to rapidly progressive ACTH-independent liver metastasis. Results. Histopathology and IHC revealed two distinct and intermingled cellular patterns, while some cells immunostained for both medullary and cortical markers. Demonstration of all stem cell biomarkers by RT/PCR and immunofluorescence was predominantly localized to the nucleus, whereas SOX2 immunoreactivity was evident in the cytoplasm as well. Conclusion. The expression of cancer stem cell biomarkers points towards the involvement of primitive embryonic cells as the origin of this neoplasm and maybe to the clinically aggressive and biochemically changing behavior.

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Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

From ACTH-Dependent to ACTH-Independent Cushing’s Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

Ramírez‐Rentería

Espinosa-de-los-Monteros

Espinosa-Cárdenas

et al. 2020

Case Reports in Endocrinology

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Undifferentiated pleomorphic sarcoma of the retroperitoneum mimicking a cortisol‐ and catecholamine‐secreting adrenal tumor

2022

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Introduction Retroperitoneal tumors with endocrine abnormalities are suspected to be functional adrenal tumors. Retroperitoneal soft tissue sarcomas are rare tumors, without endocrine potential. Case presentation A 60‐year‐old male was referred for a 15 cm mass in the left suprarenal space. His plasma cortisol and catecholamine levels were elevated. He underwent open left adrenalectomy with radical nephrectomy and his endocrinological abnormalities were improved. Pathological findings suggested that it had originated from the retroperitoneal fat tissue, and a diagnosis of undifferentiated pleomorphic sarcoma was made based on the results of immunohistochemical analysis and fluorescence in situ hybridization. Interestingly, neither cortisol nor catecholamine was elevated when, 6 months after surgery, local recurrence developed. Conclusion This is the first reported case of undifferentiated pleomorphic sarcoma accompanied by high levels of cortisol and catecholamine. We should keep in mind the possibility of tumors like retroperitoneal soft tissue sarcomas inducing endocrine abnormalities.

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Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

et al. 2021

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Background Mixed cortico-medullary adrenal carcinoma (MCMAC) is an extremely rare entity with scarce literature on its cytomorphology. Case presentation A 2-year-old girl presented with abdominal pain for 3 days and a past history of fever with significant weight loss. On examination, a non-tender left hypochondrial firm mass and an enlarged left supraclavicular node were found. Twenty-four-hour urinary levels of VMA were marginally high. Contrast-enhanced computed tomography of the abdomen showed a suprarenal heterogeneous mass encasing major vessels. Aspiration cytology of both mass and node showed similar features comprising a predominant population of singly scattered large cells with moderate cytoplasm, eccentric nucleus and prominent nucleolus in a necrotic background. Tumour cells expressed Synaptophysin and Melan-A. In view of increasing respiratory distress, debulking surgery was performed, and histopathology of the specimen revealed the presence of both malignant medullary and cortical components supported by immunohistochemistry making a final diagnosis of MCMAC. The patient succumbed to death in the postoperative period. The cytology slides were reviewed and were seen to show a dual cell population. Conclusion Coexistent malignant cortical and medullary tumour of the adrenal gland is the first case reported in the paediatric age group in the literature with only three previous case reports in adults.

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Mixed corticomedullary adrenal carcinoma – case report: Comparison in features, treatment and prognosis with the other two reported cases

Cited by 9 publications

References 17 publications

From ACTH-Dependent to ACTH-Independent Cushing’s Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

From ACTH-Dependent to ACTH-Independent Cushing’s Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

Undifferentiated pleomorphic sarcoma of the retroperitoneum mimicking a cortisol‐ and catecholamine‐secreting adrenal tumor

Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

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