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An Unusual Case of Adrenocortical Carcinoma with Multiple Facets

Tan JE, Tan FHS, Kuan YC, Chan PL, Yusri Y

J ASEAN Fed Endocr Soc, 2024;39(2):92-96.
PMID: 39620178 DOI: 10.15605/jafes.039.02.16

Adrenocortical carcinoma (ACC) is a rare malignant tumour from the adrenal cortex. Half of the cases are functional, with ACTH-independent autonomous cortisol production being the most common. It is rare for ACC to present with markedly elevated metanephrine levels, characteristic of pheochromocytoma. We report a case of a large functioning adrenal tumour with overlapping biochemical features of ACC and pheochromocytoma. Biopsy confirmed the histopathological diagnosis of metastatic ACC.

Matched MeSH terms: Adrenal Gland Neoplasms/pathology
A radiological and histological comparison of two unusual adrenal incidentalomas

Loong S, Selvarajan S, Khor LY

Malays J Pathol, 2019 Dec;41(3):327-331.
PMID: 31901917

INTRODUCTION: The increasing use of radiological imaging studies has given rise to 'incidentalomas'.
CASE REPORT: We describe two unusual and diverse incidental adrenal gland lesions, an adenomatoid nodule and a mature ganglioneuroma. Both are deemed 'indeterminate' on radiological assessment. On histology, an adenomatoid nodule is composed of variably-dilated thin-walled cysts lined by bland flattened cells and solid areas of tubules lined by eosinophilic cells with plump nuclei and prominent nucleoli. The lining cells are immunoreactive for calretinin and WT1 while negative for CK5/6, ERG and CD31. Mature ganglioneuroma features fascicles of bland spindle cells with intermixed mature ganglion cells disposed within a background myxoid stroma with no immature neuroblastic component. These spindled Schwann cells are S100 positive.
DISCUSSION: Both adenomatoid nodule and mature ganglioneuroma are rare benign adrenal tumours that need to be differentiated from other, more common adrenal lesions. The management of adrenal incidentalomas is challenging. Surgical excision is indicated if an adrenal incidentaloma is more than 4 cm in size, shows malignant features on imaging or evidence of hormone excess.

Matched MeSH terms: Adrenal Gland Neoplasms/pathology
Unusual Bone Superscan, MIBG Superscan, and 68Ga DOTATATE PET/CT in Metastatic Pheochromocytoma

Tan TH, Wong TH, Hassan SZ, Lee BN

Clin Nucl Med, 2015 Nov;40(11):867-8.
PMID: 26252329 DOI: 10.1097/RLU.0000000000000920

A 17-year-old adolescent boy with biochemically raised 2-hour urinary metanephrine and normetanephrine as well as CT findings of retroperitoneal soft tissue mass and bony metastases was referred for further assessment. Apart from Ga DOTATATE PET/CT evaluation, pretargeted systemic radionuclide therapy assessment with I-MIBG scintigraphy showed unusual phenomenon of MIBG superscan. Postsurgically, restaging Tc-MDP bone scintigraphy showed typical bone superscan features. The MIBG superscan was better delineated on post-I-MIBG therapy images.

Matched MeSH terms: Adrenal Gland Neoplasms/pathology
Fulltext Pregnancy, Primary Aldosteronism, and Adrenal CTNNB1 Mutations

Teo AE, Garg S, Shaikh LH, Zhou J, Karet Frankl FE, Gurnell M, et al.

N Engl J Med, 2015 Oct 08;373(15):1429-36.
PMID: 26397949 DOI: 10.1056/NEJMoa1504869

Recent discoveries of somatic mutations permit the recognition of subtypes of aldosterone-producing adenomas with distinct clinical presentations and pathological features. Here we describe three women with hyperaldosteronism, two who presented in pregnancy and one who presented after menopause. Their aldosterone-producing adenomas harbored activating mutations of CTNNB1, encoding β-catenin in the Wnt cell-differentiation pathway, and expressed LHCGR and GNRHR, encoding gonadal receptors, at levels that were more than 100 times as high as the levels in other aldosterone-producing adenomas. The mutations stimulate Wnt activation and cause adrenocortical cells to de-differentiate toward their common adrenal-gonadal precursor cell type. (Funded by grants from the National Institute for Health Research Cambridge Biomedical Research Centre and others.).

Matched MeSH terms: Adrenal Gland Neoplasms/pathology
Fulltext Dopamine-secreting phaeochromocytomas and paragangliomas: clinical features and management

Foo SH, Chan SP, Ananda V, Rajasingam V

Singapore Med J, 2010 May;51(5):e89-93.
PMID: 20593136

Most functional phaeochromocytomas/paragangliomas produce noradrenaline and/or adrenaline. Those that produce dopamine are rare. We describe the distinguishing clinical features of dopamine-secreting phaeochromocytomas and paragangliomas from those that secrete noradrenaline/adrenaline and the impact on their management. We present a case of a dopamine-secreting paraganglioma from our institution and review 14 case reports of dopamine-secreting phaeochromocytomas/paragangliomas published between 1984 and 2008. As observed in the literature, 80% of the tumours were extra-adrenal. Most patients presented with non-specific symptoms or mass effect without the classical presentation of catecholamine excess. The majority were diagnosed with urinary or plasma dopamine. Five patients had malignant tumours and 12 patients underwent surgical resection of the primary tumours. Unlike noradrenaline/adrenaline-secreting phaeochromocytomas/paragangliomas, dopamine-secreting tumours lack a classical presentation, are extra-adrenal and have a higher malignant potential. A routine inclusion of urinary or plasma dopamine as part of catecholamine screening in all suspected phaeochromocytomas and paragangliomas is recommended.

Matched MeSH terms: Adrenal Gland Neoplasms/pathology
Fulltext Presenting features and treatment outcome of 78 Malaysian children with neuroblastoma

Ng SM, Abdullah WA, Lin HP, Chan LL

Southeast Asian J Trop Med Public Health, 1999 Mar;30(1):149-53.
PMID: 10695803

To study the distribution of presenting features and their prognostic significance in neuroblastoma treated in a single institution in Malaysia. A retrospective study was made of 78 neuroblastoma cases diagnosed and treated in the University Hospital, Kuala Lumpur, Malaysia between June 1982 and February 1997. Diagnosis was established by standard histological criteria. The presenting features were evaluated for their distribution and prognostic influence. Disease-free survival from diagnosis was the outcome variable of interest. The ages ranged from 0.1 to 11 years old (median: 3 years old). The tumor originated from the adrenal glands in 83% and the majority of cases presented in advanced stage (stage III 22%, stage IV 66%). Bone marrow was the commonest site of distant metastasis occurring in 45% of patients. The main presenting signs and symptoms in decreasing order were pallor, fever, abdominal mass, weight loss, and bone/joint pain. Univariate analysis conferred age, initial stage and Hb level as significant prognostic factors. No influence in disease-free survival was found for sex, race, primary site, urinary vanillylmandelic acid level, white cell count and platelet count. Overall 2-year disease-free survival was achieved in 27 (39%) patients. Four patients underwent bone marrow transplant, three of whom achieved 2-year disease-free survival. The results suggest that age, initial stage and hemoglobin level are significant prognostic factors based on univariate analysis. In addition, more Malaysian children presented with adrenal primary site and advanced disease compared to previous reported studies.

Matched MeSH terms: Adrenal Gland Neoplasms/pathology