Cystic phaeochromocytoma is a distinctive subgroup with special clinical, imaging and histological features that might mislead the diagnosis

Abstract

OBJECTIVE To report and analyse cases of cyctic phaeochromocytoma at our institution and in previous publications, as adrenal cystic masses are usually associated with nonfunctional lesions, but they can be phaeochromocytoma. PATIENTS AND METHODS The prospective adrenalectomy database at our institution was reviewed to identify patients with phaeochromocytoma and cystic lesions. The clinical, radiological and histological features of cystic phaeochromocytoma were evaluated. Other previously published data were identified and compared with the present series. RESULTS In all, there were 107 adrenalectomies at our institution from November 2000 to March 2007; 31 of these patients had a confirmed diagnosis of phaeochromocytoma and six (19%) were cystic phaeochromocytomas. Three of the six cases were asymptomatic and had a negative biochemical evaluation (one male and five females, mean tumour size 6.6 cm); the imaging diagnosis was mainly based on computed tomography and magnetic resonance imaging, and the foremost feature was the enhancement of the tumour rim associated with a central cystic mass. Nine other previously published cases were reviewed. CONCLUSIONS From the present large series and previous anecdotally reported cases, patients with cystic phaeochromocytomas are more likely to be asymptomatic, to complain of chronic abdominal pain, and to have a negative biochemical evaluation, which might deter physicians from conducting a full evaluation for phaeochromocytoma and mislead the final diagnosis. As such, there could be an even higher prevalence of this entity that is yet to be determined.

Cystic Pheochromocytoma Discovered as an Adrenal Incidentaloma

“…However, it may be that the active catecholamines are converted into inactive metabolites within the tumor, and baroreceptor function decreases with age [12]. Although previous reports suggest that cystic or incidental pheochromocytoma may have non-specific abdominal symptoms [4,12], the present patient did not have any such symptoms. CT and MRI are commonly used modalities for studying the adrenal gland.…”

“…Cystic pheochromocytoma is rare, and its formation process might be intralesional hemorrhage, necrosis, and liquefaction. Patients with cystic pheochromocytomas are more likely to be asymptomatic and to have a negative biochemical evaluation than those with solid tumors [4,5], which may lead to misdiagnosis. The reason why the present patient had no specific signs or symptoms despite the elevated levels of plasma catecholamines is unclear.…”

“…The typical imaging characteristics of pheochromocytoma are a round or oval, well-circumscribed, homogeneous or heterogeneous soft-tissue mass measuring over 3 cm, increased attenuation on unenhanced CT, prominent vascularity of the mass, delayed washout of contrast media, and an high signal intensity on T 2 -weighted MRI [3,8]. In contrast, the CT features of cystic pheochromocytomas have been reported to be a relatively thick wall, the presence or absence of septa within the mass, and with persistent wall enhancement after the administration of contrast media [4], which is compatible with the findings in the present patient. The surgical indications for the removal of an incidentally discovered adrenal mass have been published [13], but the indications for the surgical resection of purely cystic adrenal masses are less clear [9].…”

“…The surgical indications for the removal of an incidentally discovered adrenal mass have been published [13], but the indications for the surgical resection of purely cystic adrenal masses are less clear [9]. Unless surgically removed, pheochromocytomas can lead to hypertensive crises and several cardiovascular complications [4]. Therefore, surgical excision is thought to be the most definitive treatment of pheochromocytomas regardless of whether the tumor is cystic or not.…”

“…The majority of previously reported adrenal cysts have been nonfunctioning and asymptomatic [2]. However, a few cases of cystic pheochromocytomas, which are typically solid tumors, have been described [2][3][4][5][6][7][8][9][10]. We describe herein a case of cystic pheochromocytoma that was discovered as an adrenal incidentaloma.…”