Results of Surgery for a Compound Adrenal Tomor Consisting of Pheochromocytoma and Ganglioneuroblastoma in an Adult. 5-year Follow-up.

Fujiwara, Takuya; Kawamura, Minoru; Sasou, Shunichi; Hiramori, Katsuhiko

doi:10.2169/internalmedicine.39.58

Cited by 22 publications

(16 citation statements)

References 20 publications

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“…Clinicopathological data of the patients are summarized in Table 1. Some data from these patients have previously been reported (13,14). All patients with composite pheochromocytoma are well without recurrence or metastasis after operation.…”

Section: Composite Pheochromocytomamentioning

confidence: 72%

Neurofibromin and NF1 Gene Analysis in Composite Pheochromocytoma and Tumors Associated with von Recklinghausen's Disease

Kimura

Watanabe

Fukase³

et al. 2002

Modern Pathology

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Composite tumor of pheochromocytoma and neuroblastoma, or ganglioneuroma, or ganglioneuroblastoma (composite pheochromocytoma), also known as mixed neuroendocrine and neural tumor, are sometimes combined with neurofibromatosis type 1 (NF1). To better understand the relationship between NF1 and composite pheochromocytoma, an immunohistochemical study using anti-neurofibromin that is an NF1 gene product and DNA sequence of NF1 Exon 31 were carried out in five cases of composite pheochromocytoma and in various tumors from five patients with NF1. Neurofibromin was not expressed in Schwann cells and sustentacular cells of composite pheochromocytomas and was very weakly or negatively expressed in neurofibroma of NF1 patients. However, it was strongly expressed in ganglionic cells and pheochromocytoma cells of the composite pheochromocytomas and also in mucosal ganglioneuromas, a gangliocytic paraganglioma, and in pheochromocytomas from the patients with NF1. Although there was no mutation in NF1 Exon 31, it could not be ruled out that there were mutations in other sites of the NF1 gene. Neurofibromin insufficiency may induce abnormal proliferation of Schwann cells in composite pheochromocytomas as well as in neurofibromatosis.

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Section: Composite Pheochromocytomamentioning

confidence: 72%

Neurofibromin and NF1 Gene Analysis in Composite Pheochromocytoma and Tumors Associated with von Recklinghausen's Disease

Kimura

Watanabe

Fukase³

et al. 2002

Modern Pathology

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“…CP can cause symptoms related to hormonal secretion by one or both components of the tumor [14]. Elevated plasma or urinary dopamine or homovanillic acid (HVA) in a patient with suspected pheochromocytoma may suggest the presence of an additional neural component [15]. However, HVA levels are influenced by the size of the neuroblastoma and there are similarities between markers.…”

Section: Discussionmentioning

confidence: 99%

“…Metastases from both components are reported, but mainly from the neural component [14]. Overall, the malignancy rate for CP is approximately 25% [2] and due to the risk of recurrence and metastasis, the presence of neuroblastoma is an indicator of poor prognosis; however, the presence of neural components does not always imply a poor prognosis [2,15].…”

Section: Discussionmentioning

confidence: 99%

An Unusual Case of a Composite Pheochromocytoma With Neuroblastoma

Steen

2014

J Endocrinol Metab

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Composite pheochromocytoma (CP) is a rare tumor of the adrenal medulla, consisting of neuroendocrine and neural components. Despite similar neural crest origins, pheochromocytomas and neurogenic tumors are distinct entities. Symptoms may arise from hypersecretion of hormones by either component; however, not all patients present with classic symptoms. Moreover, various medical conditions and substances can confound screening tests and complicate the diagnosis. The patient, a 46-year-old male, was seen in the endocrine clinic regarding a 2-year history of paroxysmal headaches, palpitations and diaphoresis. His medical history was significant for hypothyroidism and substance misuse (nicotine, marijuana and cocaine). Family history was negative for pheochromocytoma, hyperparathyroidism or thyroid malignancies. He was found to be hypertensive in clinic, and two cafe-au-lait spots and bilateral axillary freckles were noted on dermatological examination. Pheochromocytoma was suspected and investigations revealed 24-h urinary catecholamines, metanephrines and vanillylmandelic acid levels that were substantially elevated. He was asked to repeat the urine collection while abstaining from cocaine, given the potential confounding with cocaine-induced elevations of catecholamines and their metabolites; however, the patient did not comply with this. His plasma free metanephrines and chromogranin A were later found to be elevated. Abdominal computed tomography revealed a 5.8 × 5 cm mass in the left adrenal gland, with an attenuation of 40 Hounsfield units. He underwent an uncomplicated laparoscopic left adrenalectomy, after which his blood pressure normalized. Histological features of the tumor revealed findings consistent with a typical pheochromocytoma with neuroblastomatous infiltrates comprising < 5% of the tumor. The mitosis karyorrhexis index was low and there was no ganglioneuromatous infiltrate evident. Medical oncology was consulted in view of the histopathological findings, and no adjuvant therapy was recommended. Unfortunately, he did not fully comply with repeat imaging or biochemical evaluation. Six months postoperatively, the patient remains completely asymptomatic and normotensive. Cocaine-induced catecholamine effects can clinically mimic pheochromocytoma, thereby complicating the diagnosis. In addition, the biologic behavior of CP with neuroblastoma is uncertain and warrants oncologic assessment and surveillance.

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“…Preoperatively, elevation in serum or urine dopamine or homovanillic acid should raise concern that the tumor contains immature neural elements and may thus be prone to recurrence and metastases. 10 DNA ploidy analysis has been shown to have variable utility in predicting the behavior of pure pheochromocytomas. In general, however, malignant tumors were usually aneuploid or nondiploid, and diploid tumors were usually benign.…”

Section: Discussionmentioning

confidence: 99%

Incidental Finding of Composite Pheochromocytoma-Ganglioneuroma: Successful Management after Emergent Appendectomy and Review of the Literature

Royer¹,

Miron²,

Kirby³

et al. 2011

WJOES

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Composite pheochromocytoma-ganglioneuromas are rare adrenal tumors. They may be functional and while mostly sporadic, they may be associated with several genetic syndromes. Herein, we present the youngest reported patient with this tumor, which was incidentally discovered by CT scan performed prior to emergent appendectomy. The tumor was functional and she underwent a successful laparoscopic adrenalectomy with resolution of her biochemical abnormalities. Surgeons should be aware of this rare entity due to its uncertain long-term prognosis.

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Results of Surgery for a Compound Adrenal Tomor Consisting of Pheochromocytoma and Ganglioneuroblastoma in an Adult. 5-year Follow-up.

Cited by 22 publications

References 20 publications

Neurofibromin and NF1 Gene Analysis in Composite Pheochromocytoma and Tumors Associated with von Recklinghausen's Disease

Neurofibromin and NF1 Gene Analysis in Composite Pheochromocytoma and Tumors Associated with von Recklinghausen's Disease

An Unusual Case of a Composite Pheochromocytoma With Neuroblastoma

Incidental Finding of Composite Pheochromocytoma-Ganglioneuroma: Successful Management after Emergent Appendectomy and Review of the Literature

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