Oncocytic Adrenocortical Carcinoma Research Articles

Immunohistochemical characterization of a steroid secreting oncocytic adrenal carcinoma responsible for paraneoplastic hyperparathyroidism.

We report a unique case of a 44-year-old man with paraneoplastic hyperparathyroidism due to an oncocytic adrenocortical carcinoma (stage pT3N0R0M0, ENSAT 2 with a 4% Ki-67). Paraneoplastic hyperparathyroidism was associated with mild ACTH-independent hypercortisolism and increased estradiol secretion responsible for gynecomastia and hypogonadism. Biological investigations performed in blood samples from peripheral and adrenal veins revealed that the tumor secreted PTH and estradiol. Ectopic PTH secretion was confirmed by abnormally high expression of PTH mRNA and clusters of PTH immunoreactive cells in the tumor tissue. Double-immunochemistry studies and analysis of contiguous slides for the expression of PTH and steroidogenic markers (SRB-1, 3β-HSD and aromatase) were performed. The results suggested the presence of two tumor cells subtypes with large cells with voluminous nuclei producing only PTH and that were distinct from steroid producing cells.

ODP049 Oncocytic adrenocortical carcinoma misdiagnosed as renal oncocytoma and use of urinary steroid profiling in disease monitoring after mitotane therapy

Abstract Introduction Oncocytic adrenocortical carcinoma (OAC) is a rare, more indolent variant of adrenocortical carcinoma (ACC). With fewer than 70 cases reported in the English literature, the biological behavior and its response to treatment are poorly characterized. The distinction of OAC from other oncocytic neoplasms (e. g., kidneys) and variants of renal cell carcinoma (RCC) may pose diagnostic challenge pathologically. Urine steroid profiling (USP) of OAC has not been previously reported. Case description A 65-year-old Chinese man was incidentally found to have a large left upper abdominal mass in September 2014. A CT urogram showed a 15×13×13cm solid mass in the upper pole of left kidney. Left radical nephrectomy and splenectomy was performed. Pathological examination of the 'nephrectomy' showed oncocytoma. He was managed as having a renal oncocytoma with complete resection with yearly follow-up. In December 2018, a surveillance chest X-ray showed multiple lung nodules over right lung base. PET-CT in June 2019 showed bilateral hypermetabolic lung nodules suggestive of lung metastases. CT guided fine needle aspiration biopsy of lung nodule showed oncocytoma. Pathology review of both the lung and 'nephrectomy' biopsy revealed identical cores of oncocytic tumor cells while immunohistochemical studies displayed positivity for SF-1 and Melan-A suggestive of adrenocortical origin. In the presence of lung metastases, a revised diagnosis of OAC was made with the original tumor classified as oncocytic adrenocortical neoplasm of uncertain malignant potential by the Lin-Weiss-Bisceglia system. Hormonally the tumor was non-functional with suppressible cortisol post 1mg overnight dexamethasone suppression test and normal renin, aldosterone, estradiol and DHEA-S levels. Twenty-four-hour urine steroid profiling showed elevated tetrahydro-11-deoxycortisol 788ug/day [RR 10-90µg /day], pregnanediol 374µg /day [RR 41-348µg/day] and pregnenetriol 487µg /day [RR 25-256µg /day]. Mitotane was started and titrated up to 1500mg daily, aiming at a serum mitotane level of 14- 20µg /ml. Resolution of lung metastases was noted 6 months later and the abnormal urinary steroid metabolites were also normalized. The patient remained in disease remission 18 months after commencement of mitotane. Conclusion Oncocytic adrenocortical carinoma may be mistaken as other oncocytic neoplasms or variants of RCC pathologically and is classified by the Lin-Weiss-Bisceglia system. An accurate diagnosis has significant impact on disease prognostication and management. OAC may respond more favorably to mitotane monotherapy in comparison to typical ACC. Urine steroid profiling is potentially useful in the surveillance and disease monitoring of OAC. Reference: (1) Harada et al. A rare case of oncocytic adrenocortical carcinoma clinically presented as an incidentaloma. Endocr J. 2020 Aug 28;67(8): 883-888. Presentation: No date and time listed

PSAT025 Functional Adrenocortical Oncocytic Neoplasm: A Rare Cause of ACTH Independent Cushing's Syndrome

Abstract Background Adrenal oncocytomas (AOCs) or adrenocortical oncocytic neoplasms (ACONs) are extremely rare tumors of adrenal glands characterized by the presence of oncocytes, which are epithelial cells with eosinophilic cytoplasm. Only about 140 cases have been reported in literature so far. AOCs are more common in females (2.5: 1), more prevalent on the left adrenal gland (3.5: 1), and tumor size ranges from 3 to 17 cm. About two thirds of AOCs are reported to be nonfunctioning, and only less than 6% of functioning are cortisol secreting. The malignant potential of these tumors has been described quite variably in current literature. Based on the most recent systematic literature review, about 35% were found to be benign, 24% malignant, and 41% borderline, based on the Lin Weiss Bisceglia system. The mainstay of treatment is surgical resection. Here we describe a case where biochemical workup and pathology revealed a functional AOC with uncertain malignant potential. Clinical Case A 68 year old female with hypertension was found to have an incidental left 3 cm adrenal nodule on CT abdomen and pelvis during a workup for hematuria. The nodule was biopsied and initially found to be benign. Afterwards, she was referred to endocrinology where biochemical testing was consistent with ACTH-independent Cushing's syndrome. She had three elevated late night salivary cortisol - 0.10 mcg/dL, 0.14 mcg/dL, and 0.19 mcg/dL (≤0. 09 mcg/dL), three abnormal 1 mg dexamethasone suppression tests - 16.4 mcg/dL, 15.1 mcg/dL, and 17.1 mcg/dL (<1.8 mcg/dL), two normal 24 hour urinary cortisol - 22.5 and 31.5 mcg/24h (4-50 mcg/24h), and ACTH <5 pg/mL (6-50 pg/mL). She was then referred to surgery and was treated by laparoscopic adrenalectomy. Surgical pathology showed a 3.5 cm adrenal cortical tumor with oncocytes infiltrating into the periadrenal adipose tissue, with positive staining for synaptophysin and negative for P53. On Ki67 staining, some hot spots were greater than 5%. Therefore, the tumor was characterized as AOC with undetermined malignant potential or a low grade, oncocytic adrenocortical carcinoma. She was evaluated by oncology and radiation oncology, and it was decided to monitor the patient without systemic therapy or adjuvant radiation therapy. A surveillance approach was favored given that the risks of adjacent bowel and kidney toxicity outweighed the benefits of radiation. Conclusion We hereby report a case of AOC with undetermined malignant potential. This case highlights many challenges in the diagnosis, management and long-term surveillance of patients with AOCs, and need for further research. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

ODP050 Oncocytic Adrenocortical Carcinoma Presenting with Hematuria

Abstract Introduction Adrenocortical carcinoma (ACC) is rare and aggressive endocrine tumor. It is commonly noticed due to symptoms of hormone excess, mass-effect, or incidentalomas. Different histological variants are noted, which include: oncocytic, myxoid, sarcomatoid, and the conventional subtype. The oncocytic variant is usually seen on the left side, and usually presents with symptoms of mass-effect. Only around 17% were noted to be functional. We present an interesting case of ACC (oncocytic type) which presented with hematuria. Case Presentation 70-year-old female with a history of morbid obesity, hypertension, anxiety, GERD, tubular adenoma, hyperlipidemia, and lumbar radiculopathy came in to see her primary care for hematuria and back pain. She has a long-standing history of back pain, for which she has had an EMG, which showed mild axonal polyneuropathy and bilateral L5 radiculopathy. Apart from hematuria, she denies any other urinary complaints. Additionally, she denied any fever, chills, nausea, abdominal pain, sweating, tremor, headache, palpitations, easy bruising, fractures, muscle weakness, hirsutism, or changes in her weight. She has a family history of pancreatic cancer and is currently retired, and denies alcohol, tobacco, or recreational drug use. A KUB X-ray was ordered which showed calcification in the left upper quadrant, concerning for a splenic artery aneurysm; this led to a CTA abdomen, which showed a large 7.2 cm heterogenous enhancing solid left adrenal mass, concerning for primary adrenal carcinoma, which was new compared to imaging 2 years ago. Also seen was a 0.7 cm enhancing right hepatic lobe lesion, along with a 1.5 cm splenic artery aneurysm. Following this, an abdomen MRI with contrast showed a 7×7×7 cm solid heterogeneously enhancing left upper quadrant mass beside the left adrenal gland and pancreas. Subsequently, a CT guided needle biopsy was done, which revealed scant adrenal tissue. PET scan showed a hypermetabolic mass, arising from the left adrenal, consistent with adrenal carcinoma. At this point, an endocrinology referral was placed, and other lab work including 24-hour urine free cortisol, DHEA-S, 24-hour urine metanephrine, and aldosterone/renin ratio was done. DHEA-S level was elevated at 264 mcg/dl (12-133). She was then transferred to a tertiary care facility, where she underwent a left adrenalectomy, para-aortic lymphadenectomy, nephrectomy, splenectomy, and distal pancreatectomy. Pathology confirmed adrenal cortical carcinoma (oncocytic type), with negative margins, Ki-67 labelling index of 25%, and no lymphovascular invasion, pT2pNO. She was then discharged home with visiting nurses. Conclusion Oncocytic ACC is an infrequent tumor, which presents with symptoms of mass effect, and definitive diagnosis requires histopathological assessment. The Lin–Weiss–Bisceglia (LWB) system and Helsinki score help establish malignant potential. Fortunately, it has a decreased tendency to metastasize and a better prognosis than other ACC subtypes. Further studies to understand oncocytic ACC are needed. Presentation: No date and time listed

Differences in Clinicopathologic Behavior of Oncocytic Adrenocortical Neoplasms and Conventional Adrenocortical Carcinomas.

Oncocytic adrenocortical neoplasms (OANs) are rare endocrine tumors that present as a spectrum from benign to malignant. The outcomes after surgical resection of the oncocytic variant of adrenocortical carcinoma remain poorly understood. We sought to characterize the clinicopathologic features of OAN and compare oncocytic adrenocortical carcinoma (OAC) with conventional adrenocortical carcinoma (ACC). Adult patients who underwent adrenalectomy for OAN or ACC between January 1990 and September 2020 were identified. Demographics, clinicopathologic factors, American Joint Committee on Cancer stage, and cancer-related outcomes were reviewed. A matched cohort analysis of disease-free survival (DFS) and overall survival (OS) was performed between patients with OACs and those with ACCs. Forty-one patients with OAN and 214 patients with ACC were included. The OAN cohort median age was 45.2 years [interquartile ratio (IQR) 38.5-54.0 years], and 61.0% were female. OANs were benign (n = 11), of uncertain malignant potential (UMP, n = 9), or OAC (n = 21). Disease recurrence occurred in 12 (57.1%) patients with OAC compared with 1 (11.1%) and 0 patients with UMP or benign OAN, respectively (p < 0.001). Seven (33.3%) patients with OAC died during follow-up compared with 0 patients with UMP or benign OAN (p = 0.020). Kaplan-Meier survival analysis found no difference in DFS between ACC and OAC groups before (p = 0.218) and after 2:1 matching (p = 0.417). Overall survival was shorter for patients who had ACC compared with those who had OAC (p = 0.031), but the difference was not evident with matched analysis (p = 0.200). OAN presents as a spectrum from benign indolent tumors to aggressive carcinomas. OACs demonstrate similar clinicopathologic behavior and recurrence-free and overall survival when matched to conventional ACCs.

313 Importance of Surgical Approach and Resection Status on Outcomes of Patients with Adrenocortical Carcinoma (ACC)

Abstract Aim Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with overall poor prognosis. We aimed to examine the relationship between tumour resection margins (R0/R1), surgical approach (laparoscopic vs. open) and clinical outcomes in our practice. Method We conducted a retrospective review of patients diagnosed with histology-proven ACC (01/2015–12/2020) who proceeded to surgery and were followed up at Addenbrooke’s hospital (n = 24). We examined overall and progression-free survival (PFS) censored at date of death or at last follow-up. Results Patients had a median(SD) age at presentation of 58.5(11.0), were female in 62.5%; median(SD) length of follow-up was 29(20.1) months. The tumour histology was oncocytic variant in 50% of cases. R0 resection was achieved in 16/23 cases and was associated with better PFS [median(SD): 28.5(19.3) months for R0 vs. 8(25.0) for R1] and overall survival [median(SD): 33(18.4) months for R0 vs. 29(22.6) for R1]. 15/16 and 1/7 patients with R0 and R1 resection respectively were alive at end of follow-up. R1 resection status and survival were associated with ENSAT stage (85.7% stage III-IV for R1 vs. 25.1% for R0) although tumour size was not significantly different. A greater proportion of R1 patients (57.1%) had laparoscopic surgery (3 of those had tumour size&amp;gt;6cm) than R0 patients (25%). Outcomes for oncocytic ACC tumours were not different. Conclusions R0 resection is of paramount importance for ACC outcomes. Careful pre-operative work-up of indeterminate adrenal lesions to raise suspicion for ACC and consideration of open surgery for large tumours may help improve outcomes.

American Association of Clinical Endocrinology Disease State Clinical Review on the Evaluation and Management of Adrenocortical Carcinoma in an Adult: a Practical Approach

The aim of this Disease State Clinical Review is to provide a practical approach to patients with newly diagnosed adrenocortical carcinoma, as well as to follow-up and management of patients with persistent or recurrent disease. This is a case-based clinical review. The provided recommendations are based on evidence available from randomized prospective clinical studies, cohort studies, cross-sectional and case-based studies, and expert opinions. Adrenocortical carcinoma is a rare malignancy, often with poor outcomes. For any patient with an adrenal mass suspicious for adrenocortical carcinoma, the approach should include prompt evaluation with detailed history and physical exam, imaging, and biochemical adrenal hormone assessment. In addition to adrenal-focused imaging, patients should be evaluated with chest-abdomen-pelvis cross-sectional imaging to define the initial therapy plan. Patients with potentially resectable disease limited to the adrenal gland should undergo en bloc open surgery by an expert surgeon. For patients presenting with advanced or recurrent disease, a multidisciplinary approach considering curative repeat surgery, local control with surgery, radiation therapy or radiofrequency ablation, or systemic therapy with mitotane and/or cytotoxic chemotherapy is recommended. As most health care providers will rarely encounter a patient with adrenocortical carcinoma, we recommend that patients with suspected adrenocortical carcinoma be evaluated by an expert multidisciplinary team which includes clinicians with expertise in adrenal tumors, including endocrinologists, oncologists, surgeons, radiation oncologists, pathologists, geneticists, and radiologists. We recommend that patients in remote locations be followed by the local health care provider in collaboration with a multidisciplinary team at an expert adrenal tumor program. ACC = adrenocortical carcinoma; ACTH = adrenocorticotropic hormone; BRACC = borderline resectable adrenocortical carcinoma; CT = computed tomography; DHEAS = dehydroepiandrosterone sulfate; EDP = etoposide, doxorubicin, cisplatin; FDG = 18F-fluorodeoxyglucose; FNA = fine-needle aspiration; HU = Hounsfield units; IVC = inferior vena cava; LFS = Li-Fraumeni syndrome; MEN1 = multiple endocrine neoplasia type 1; MRI = magnetic resonance imaging; OAC = oncocytic adrenocortical carcinoma; PC = palliative care; PET = positron emission tomography.

SUN-179 Oncocytic Adrenocortical Carcinoma: A Case Report

Adrenocortical carcinoma (ACC) is a rare tumor with a poor prognosis. Oncocytic variant of adrenocortical carcinoma is considered to be an even more rare malignancy with four reported in the literature in 2002 and thirty-six documented in 2015 i ii. This case report illustrates the case of oncocytic ACC.A 40-year-old woman post-hysterectomy from cervical cancer presented to the ER with severe left flank and back pain for two weeks. There was no weight loss or early satiety. CT scan revealed a large left adrenal mass measuring 12x9x13 cm, displacing the left kidney inferiorly with no metastasis to nearby structures. We ruled out functional adrenal mass through a 24-hour urine collection and blood work, then performed open left adrenalectomy.Grossly, it was a large, tan-colored mass, weighing approximately 2,500 grams. On biopsy, we incidentally found the mass to be oncocytic adrenocortical carcinoma. Using the Lin-Weiss-Bisceglia criteria, it met two major and one minor criteria, when only one major criterion is required to indicate malignancy. Immunohistochemical stains showed that the tumor cells were positive for Mart-1, inhibin, and synaptophysin. Quantitative Ki-67 showed a proliferation index of 10% in average (>5% is considered malignant). Even though adrenocortical carcinoma, specifically the oncocytic variant of ACC, is a very rare occurrence, it should be on the differential diagnosis for a chief complaint of unilateral flank pain with a large adrenal mass on CT due to its poor prognosis.Endnotes i Hoang, M., Ayala, A. & Albores-Saavedra, J. Oncocytic Adrenocortical Carcinoma: A Morphologic, Immunohistochemical and Ultrastructural Study of Four Cases. Mod Pathol 15, 973-978 (2002) doi:10.1038/modpathol.3880638 ii Kalra S, Manikandan R, Srinivas BH. Oncocytic adrenocortical carcinoma--a rare pathological variant. BMJ Case Rep. 2015;2015:bcr2014208818. Published 2015 Apr 24. doi:10.1136/bcr-2014-208818

Oncocytic adrenocortical carcinoma

Oncocytic adrenocortical carcinoma is an extremely rare tumor, with only 150 cases reported in the literature. A 48-year-old male patient, with right adrenalectomy due to a 7-cm non-functional adrenal inciden- taloma. From a clinical imaging point of view, oncocytic adrenocortical carcinoma cannot be distinguished from other more common adrenal tumors; therefore, the diagnostic confirmation is histological. It is rarely malignant, and the accepted treatment is laparoscopic adrenalectomy. In our case, two minor histological criteria classify it as a potentially malignant tumor.

Somatostatin receptor expression in adrenocortical carcinomas

Background. Adrenocortical carcinoma (ACC) is a rare malignant tumor characterized by an annual incidence of 0.5—2 cases per million population. Surgery is the-first line treatment for ACC. When total tumor resection is not possible due to its proliferation or progression, mitotane (o,p’DDD) is used. In this case, stabilization and partial response (as assessed by RECIST criteria) was observed only in 48.7% of cases, necessitating the search for new therapeutic targets.&#x0D; Objective — the study was aimed at assessing the somatostatin receptor expression in adrenocortical carcinomas and adrenal cortex tumors with uncertain malignant potential.&#x0D; Material and methods. Surgical material from adrenocortical tumors of 13 patients (4 males and 9 females aged from 28 to 68 years) was used. In all cases, the diagnosis was verified by morphological and immunohistochemical (IHC) studies: ACC was detected in 10 cases (including 1 case of ACC liver metastasis), oncocytic carcinoma — 1 case, oncocytoma with uncertain malignant potential — 2 cases. Morphological assessment of ACC was carried out according to Weiss criteria (for ACC tumors) and Lin—Weiss—Bisceglia criteria (for oncocytic neoplasms of the adrenal cortex). IHC study was carried out with antibodies to the spectrum of adrenal cortex-specific markers, as well as Ki-67 and somatostatin receptors 2 and 5 (SSTR2 and SSTR5).&#x0D; Results. The expression of SSTR2 and/or 5 was detected in 8 (61.5%) of 13 cases of ACC. Isolated SSTR2 expression was observed in 4 cases (4/13), while SSTR5 expression was observed in 6 cases (6/10). In 2 cases (2/10), co-expression of both receptor types was observed. SSTR expression was observed both in ACC and ACC liver metastasis, as well as in oncocytic ACC.&#x0D; Conclusion. SSTR2 and/or 5 expression in ACC tissue expands diagnostic and prognostic capabilities for this pathology.

Challenges in surgical pathology of adrenocortical tumours.

Adrenocortical carcinomas are rare tumours that can be diagnostically challenging. Numerous multiparametric scoring systems and diagnostic algorithms have been proposed to differentiate adrenocortical adenoma from adrenocortical carcinoma. Adrenocortical neoplasms must also be differentiated from other primary adrenal tumours, such as phaeochromocytoma and unusual primary adrenal tumours, as well as metastases to the adrenal gland. Myxoid, oncocytic and sarcomatoid variants of adrenocortical tumours must be recognized so that they are not confused with other tumours. The diagnostic criteria for oncocytic adrenocortical carcinoma are different from those for conventional adrenocortical carcinomas. Adrenocortical neoplasms in children are particularly challenging to diagnose, as histological features of malignancy in adrenocortical neoplasms in adults may not be associated with aggressive disease in the tumours of children. Recent histological and immunohistochemical studies and more comprehensive and integrated genomic characterizations continue to advance our understanding of the tumorigenesis of these aggressive neoplasms, and may provide additional diagnostic and prognostic utility and guide the development of therapeutic targets.

Virilizing oncocytic adrenocortical carcinoma: clinical and immunohistochemical studies

Context: Oncocytic tumors of the adrenal cortex are rare, mostly nonfunctioning and benign.Setting: Report virilizing oncocytic adrenocortical carcinoma in a 50-year-old woman.Patient: She presented a recent and progressive virilization syndrome, associated with high blood pressure. Hormonal evaluation showed elevated serum testosterone and delta-4-androstenedione levels, normal urinary free cortisol level and incomplete suppression of cortisol at the 1 mg dexamethasone suppression test. CT scan of the abdomen revealed a 35 mm left adrenal mass.Intervention: The patient underwent a left adrenalectomy, and the histological study showed a 3 cm oncocytic adrenocortical carcinoma with signs of malignancy.Results: Immunohistochemical study revealed that tumor cells expressed the steroidogenic enzymes involved into androgen synthesis (3βHSD and P450c17α), P450 aromatase and luteinizing hormone (LH) receptors. Post-operatively, signs of virilization improved rapidly, serum testosterone and delta-4-androstenedione levels returned to normal, as did the dexamethasone suppression test. During follow-up CT-scan and 18-FDG PET/CT showed a right ovary mass, corresponding to a follicular cyst associated with hyperthecosis. The patient is alive with no recurrence 48 months after adrenal surgery.Conclusion: Oncocytic adrenocortical carcinomas, although extremely rare, should be considered in women with a virilization syndrome. In this woman immunohistochimical studies revealed the presence of steroidogenic enzymes involved into androgen synthesis and aromatization, and LH receptors could be implicated in this pathology.

Hormonally Active Adrenocortical Oncocytic Carcinoma Presenting with Hirsutism and Amenorrhea: A Rare Case Report and Literature Review

Hormonally Active Adrenocortical Oncocytic Carcinoma Presenting with Hirsutism and Amenorrhea: A Rare Case Report and Literature Review Thirty-seven year old female, referred to our Urology Service from Internal Medicine and General Surgery for an assessment of an injury to the left adrenal gland. The injury was discovered incidentally by abdominal imaging, as the patient had been suffering from amenorrhoea and hypertrichosis for over a year. The patient also referred to a tingling sensation in the face and neck and to occasional headaches and blurred vision, as well as weight loss during this time period. The exploration showed widespread oedema, more pronounced on the lower limbs, a marked distribution of hair over the abdomen, chest, arms, and legs, and erythematous rashes on the chest, forearms and abdomen..

Oncocytic Adrenocortical Carcinoma in a Putty-nosed Monkey (Cercopithecus nictitans) with Hyperadrenocorticism

Oncocytic adrenocortical tumours are rare in man and have never been described in non-human primates. An oncocytic adrenocortical carcinoma was identified in an 18-year-old female putty-nosed monkey (Cercopithecus nictitans) with hyperadrenocorticism and invasive aspergillosis. Microscopically, the tumour consisted of large cells with abundant eosinophilic, granular cytoplasm containing numerous mitochondria as identified by electron microscopy. Tumour cells had large nuclei with occasional intranuclear cytoplasmic pseudoinclusions. Immunohistochemically, tumour cells expressed vimentin, synaptophysin and neuron-specific enolase, while they were negative for cytokeratin, chromogranin-A, melan-A and S100.

Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants.

Adrenocortical carcinoma (ACC) is a rare, heterogeneous malignancy with a poor prognosis. According to WHO classification 2004, ACC variants include oncocytic ACCs, myxoid ACCs and ACCs with sarcomatous areas. Herein, we provide a comprehensive review of these rare subtypes of adrenocortical malignancy and emphasize their clinicopathological features with the aim of elucidating aspects of diagnostic categorization, differential diagnostics and biological behavior. The issue of current terminology, applied to biphasic tumors with pleomorphic, sarcomatous or sarcomatoid elements arising in adrenal cortex, is also discussed. We additionally present emerging evidence concerning the adrenal cortical tumorigenesis and the putative adenoma–carcinoma sequence as well.

Oncocytic adrenocortical carcinoma

Only 17 cases of oncocytic adrenocortical carcinoma have been reported in the English literature. Here, we report an incidental case of oncocytic adrenocortical carcinoma. The patient was a 69-year-old man with the chief complaint of abdominal pain. Abdominal computed tomography revealed a left adrenal tumor. No hormonal symptoms were observed. The excised tumor was whitish, encapsulated, and 75 x 60 x 45 mm in size. Large polygonal tumor cells were arranged in a generally diffuse architecture and exhibited abundant eosinophilic granular cytoplasm. Nuclear atypia with atypical mitotic figures and capsular and sinusoidal invasions were observed. The tumor cells were immunopositive for vimentin, neuron-specific enolase, and synaptophysin but not for alpha-inhibin, melan A, or p53. Diffuse and strong immunopositivity with an antimitochondrial antibody proved that this tumor was truly oncocytic. Upon review of previous cases of oncocytic adrenocortical tumors, we reconsidered the diagnostic findings of the potential for malignancy.

Adrenocortical oncocytic carcinoma with recurrent metastases: a case report and review of the literature

BackgroundAdrenal cortex oncocytic carcinoma (AOC) represents an exceptional pathological entity, since only 22 cases have been documented in the literature so far.Case presentationOur case concerns a 54-year-old man with past medical history of right adrenal excision with partial hepatectomy, due to an adrenocortical carcinoma. The patient was admitted in our hospital to undergo surgical resection of a left lung mass newly detected on chest Computed Tomography scan. The histological and immunohistochemical study revealed a metastatic AOC. Although the patient was given mitotane orally in adjuvant basis, he experienced relapse with multiple metastases in the thorax twice in the next year and was treated with consecutive resections. Two and a half years later, a right hip joint metastasis was found and concurrent chemoradiation was given. Finally, approximately five years post disease onset, the patient died due to massive metastatic disease. A thorough review of AOC and particularly all diagnostic difficulties are extensively stated.ConclusionHistological classification of adrenocortical oncocytic tumours has been so far a matter of debate. There is no officially established histological scoring system regarding these rare neoplasms and therefore many diagnostic difficulties occur for pathologists.

Functional Oncocytic Adrenocortical Carcinoma

We present a case of oncocytic adrenocortical carcinoma in a 25-year-old man who presented with persistent hypertension, hypokalemia, and a large right adrenal mass. Clinical workup revealed increased serum aldosterone level, suppressed serum ACTH level and high 24-h urine cortisol. Histologically the tumor showed several features of malignancy and electron microscopy confirmed oncocytic differentiation. This case is reported as the first case of an aldosterone and cortisol-producing malignancy with an oncocytic phenotype.

Oncocytic adrenocortical carcinoma

Oncocytic adrenocortical carcinoma is rare. To our knowledge, only 6 cases have been previously reported. We describe an additional case of oncocytic adrenocortical carcinoma. A 54-year-old man presented with a right subcostal mass. Computed tomography demonstrated a massive tumor in the right abdomen. Because renal or adrenal cancer was suspected, right adrenalectomy and nephrectomy were performed. Histologic examination revealed an oncocytic adrenocortical carcinoma. Five months postoperatively, multiple metastases had developed and were treated with surgical resection, chemotherapy, vascular embolization, and radiotherapy. At last follow-up, the patient was alive with pulmonary and adrenal metastases and undergoing treatment with mitotane.