Composite pheochromocytoma associated with neurofibromatosis type 1.

Introduction: Composite pheochromocytoma is a rare tumor, occurring in only 3% of pheochromocytomas. We report a case of composite pheochromocytoma with neurofibromatosis type 1. Case presentation: A 42-year-old man was referred to our department for further evaluation of an incidentally detected right adrenal tumor. He was a patient at another hospital for neurofibromatosis type 1. The serum and urinary catecholamine levels exceeded the normal range. Abdominal computed tomography and magnetic resonance imaging showed a 2.8 cm diameter right adrenal tumor, and 123I-metaiodobenzyguanidine scintigraphy showed radioisotope uptake. He was diagnosed with pheochromocytoma and underwent a right laparoscopic adrenalectomy. Histopathological examination revealed that the tumor consisted of a pheochromocytoma and ganglioneuroma. The final diagnosis was composite pheochromocytoma-ganglioneuroma. Five years after surgery, no recurrence was observed. Conclusion: Preoperative diagnosis of composite pheochromocytoma-ganglioneuroma is difficult; therefore, histopathological examination is necessary for a definitive diagnosis. Pheochromocytoma management requires lifelong follow-up.

Management of a Composite Pheochromocytoma (Pheochromocytoma/Neuroblastoma) in Adult Patient Recurring After Several Years: A Complex Case Report.

Pheochromocytoma/neuroblastoma composite tumors are rare entities for which little is known. We report an atypical case of a 39-year-old man with secondary bone locations of a composite tumor, 7 years after resection of adrenal neuroblastoma, with constitutional alteration of SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4 whose role is unknown. The diagnosis of a peripheral neuroblastic tumor in adulthood is difficult and even more so when it is a composite tumor. In the absence of a standard of care, management is varied and discussions about treatment modalities for these patients are complex.

Composite pheochromocytoma of the adrenal gland-a review of published cases.

Composite pheochromocytoma (CP) is a rare adrenal tumor, composed of ordinary pheochromocytoma and neuroblastic components. There is a paucity of information in the literature regarding this entity. We report the case of a 56-year-old woman with a CP of the left adrenal gland with a ganglioneuroma component. A review of the published literature found 110 cases of CP. The median age was 51.5 (5.86) years, and 59/110 (53.6%) were female. Association with genetic predisposition syndromes was found in 22/110 (20%), the most common of which was neurofibromatosis type 1, in 15/110 (13.6%). The most common histologic type of the neuroblastic component was ganglioneuroma in 83/110 (75.5%). Twenty-seven cases reported SDHB immunohistochemistry results; none of which was positive. Nine patients (8.2%) presented/developed metastatic disease, and 9 patients (8.2%) died from disease. To our knowledge, this is the largest review describing clinical, histopathological, molecular, and prognostic features of CP.

Composite pheochromocytoma with spindle cell sarcoma - a rare tumor of the adrenal gland: case report.

Composite pheochromocytomas with a non-pheochromocytoma component stemming from an embryological origin other than the neural crest comprise a unique entity. We present a patient diagnosed with a composite pheochromocytoma with spindle cell sarcoma, which is, to our knowledge, the first case reported in the current literature. A 45-year-old female patient with elevated blood pressure had undergone adrenalectomy for clinically and radiologically diagnosed pheochromocytoma. Macroscopic examination of the specimen showed an encapsulated mass containing two nodules. One tumor was composed of large polygonal neoplastic cells with round vesicular nuclei and granular cytoplasm forming the characteristic Zellballen pattern consistent with pheochromocytoma, while the other was composed of spindle cells with hyperchromatic nuclei and prominent nucleoli, thus resembling a sarcoma. The patient was discharged on the fourth post-operative day without any complications. Even though for both tumors surgical resection is the main treatment modality, our case report aims to shed light on and discuss the etiology and management of a rarely presented composite pheochromocytoma and spindle cell sarcoma.

Mixed Corticomedullary Tumor Accompanied by Unilateral Aldosterone-Producing Adrenocortical Micronodules: a Case Report.

Mixed corticomedullary tumors (MCMTs) are rare and comprise medullary and cortical cells in a single adrenal tumor. The mechanisms underlying their development have not been fully elucidated. Here, we report a case of MCMT in a 42-year-old woman. Based on the preoperative clinical findings, the patient was diagnosed as having a pheochromocytoma with subclinical Cushing syndrome. Postoperative pathological diagnosis revealed that the tumor demonstrated morphologically distinct medullary and cortical components, which produced catecholamines and cortisol, respectively. Hybrid tumor cells producing both catecholamines and cortisol were not detected. Adrenocorticotropin (ACTH)-positive tumor cells were identified to be present in the pheochromocytoma. This ectopic production of ACTH can contribute to an autonomous cortisol production in a paracrine manner. In addition, micronodules producing aldosterone were detected in the adrenal tissue adjacent to the tumor. The simultaneous development of these 2 lesions may not be correlated with each other; however, this case confirms the importance of a detailed histopathological examination of the adrenal lesions harboring complicated hormonal abnormalities by providing pivotal and indispensable information on their pathogenesis and the possible interaction of the hormones produced in the adrenal gland.

Composite Pheochromocytoma: A Rare Form of Tumor.

The pheochromocytomas are one of the rare and curable causes of secondary hypertension arising from adrenal medulla, commonly presenting with hypertension; either paroxysmal or persistent. Very rarely they may show cells belonging to more than one line of differentiation and are called as mixed or composite pheochromocytoma.

Composite Pheochromocytoma Presenting as Severe Lactic Acidosis and Back Pain: A Case Report.

Pheochromocytoma is a rare, catecholamine secreting tumor arising from chromaffin cells. Presentation of this tumor is highly variable, the most common being hypertension, tachycardia, sweating, and headache. Lactic acidosis and back pain are rare complications of this tumor. We report a 51-year-old gentleman with composite pheochromocytoma, which is rarer than pheochromocytoma, presenting as severe back pain and lactic acidosis.

Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report.

BACKGROUND: Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to be associated with pheochromocytoma/paragangliomas such as multiple endocrine neoplasia type 2 (2A or B). To the best of our knowledge, only four cases of composite pheochromocytoma/paragangliomas associated with multiple endocrine neoplasia type 2 have been reported. CASE PRESENTATION: A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years. She had a familial history of diabetes and multiple endocrine neoplasia type 2. A radiological examination revealed thyroid lesions and bilateral adrenal medulla tumors. Our patient had undergone bilateral adrenalectomy, total thyroidectomy with cervical lymphadenectomy, and parathyroidectomy. A pathological examination confirmed the multiple endocrine neoplasia type 2A consisting of left medullary pheochromocytoma, right medullary composite pheochromocytoma-ganglioneuroma, medullary carcinoma of the thyroid with lymph node metastasis and parathyroid hyperplasia. A genetic analysis also revealed that our patient had a RET germline mutation. CONCLUSION: Composite pheochromocytoma/paraganglioma associated with multiple endocrine neoplasia type 2 is a very rare occurrence, as the current literature provides only a few cases. Further reported cases are needed in order to understand the behavior and the pathogenesis of this uncommon entity.

A rare and easily misdiagnosed tumor of the urinary bladder: primary composite pheochromocytoma.

BACKGROUND: Composite pheochromocytoma, which is a tumor composed of ordinary pheochromocytoma and other components, is extremely rare in bladder. We present a case of this rarely seen tumor in bladder, and discuss the clinical features, behavior, pathologic findings, essentials of diagnosis and prognosis of this tumor after literature review. METHODS: Specimens from a 55-year-old woman with primary composite pheochromocytoma in bladder were analyzed by immunohistochemical (IHC) and fluorescence in situ hybridization (FISH). Clinicopathological characteristics were also collected and discussed. Then, we searched and reviewed literatures related with composite pheochromocytoma that were published in PubMed over the last 80 years. RESULTS: B-mode ultrasound scanner and Magnetic Resonance Imaging (MRI) detected a papillary and infiltrative tumor mass in the irregularly thickened posterior walls of the urinary bladder. Histologically, the tumor showed evidence of big ganglion-liked cells and small round cells in cystoscopy biopsy and typical "Zellballen" structure in gross specimen. On PubMed, a total of 58 cases of composite pheochromocytoma has been reported during 1933-2017. CONCLUSION: Composite pheochromocytoma in urinary bladder, which has distinctive clincopathologic features, is an extremely rare disease and can only be diagnosed by pathologists. Diagnosis is difficult before histopathological examination and should be considered in patients with no risk factors for usual bladder tumor. The rate of metastasis and death is higher in cases where the second component is not ganglioneuroma. Fortunately, treatment of this type of tumor remains the same as pheochromocytoma. Patients with localized tumors have an extremely favorable prognosis.

Diverse proportion in composite pheochromocytoma-ganglioneuroma may induce varied clinical symptom: comparison of two cases.

Composite pheochromocytoma-ganglioneuroma is extremely rare. We described two cases of composite pheochromocytomas in the adrenal medullar. Case 1 was a 70-year-old male presenting with lower abdominal pain and normal blood electrolytes. Case 2 was a 48-year-old female with palpitation and back tenderness. Biochemical investigations showed hypocalcium, hypokalemia and high level of vma. The histological images and the immunohistochemical staining demonstrated the two cases composed of pheochromocytoma and ganglioneuromoma components. Ganglioneuroma component in case 2 accounted for more proportion than that in case 1. We speculated that the varied clinical symptoms were related with the diverse proportions in composite pheochromocytome-ganglioneuroma.