chromogranin A, norâmetanephrine, metanephrine and 3âmethoxytyramine were increased. Comorbidities included: Hypothyroidism in multinodular goiter, in ...
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Very late recurrence of an apparently benign pheochromocytoma ABSTRACT Pheochromocytoma is a tumor that has the probability to relapse in about 10% of surgically treated cases. Currently, the only recognized criteria of malignancy in these neoplasms are the evidence of metastasis at non-chromaffin sites. No reliable clinical or histopathological parameter has been, so far, identified to predict malignancy in patients with diagnosis of primary pheochromocytoma. Several authors has attempted to propose morphologic features to detect potentially malignant pheochromocytomas, but there are still too many reported cases of recurrence, also after decades, in tumors that, according to the current knowledge, are considered "benign". Here we report a case of recurrence, after 25 years, of a pheochromocytoma that had not enough criteria to be considered as malignant. KEY WORDS: Follow-up, late recurrence, malignancy, pheochromocytoma
INTRODUCTION Pheochromocytomas are neuroendocrine neoplasms that have been called “10% tumors”, as they approximately are 10% malignant, 10% bilateral and 10% familial.[1] Several scoring systems, such as PASS (Pheochromocytoma of Adrenal gland Scaled Score)[2] have been proposed to assess their malignancy. Recently, De Wailly et al.,[3] attempted to define new malignancy histologic criteria that included tumor size, weight, presence of necrosis, high mitotic rate [>3/10 high power field (HPF)], ki67 index > 4%, S100 absence. Long term follow‑up is moreover recommended in patients with inherited tumors, large size or multiple localizations.[4] The WHO states that the only reliable criterion to define pheochromocytoma malignancy is the presence of loco‑regional invasion or metastasis at non‑chromaffin sites distant from the primary tumor.[1] However, several cases of relapse, some after decades from the primary diagnosis, have been reported even in patients with no one of the proposed prognostic indicators.[2,5‑7] CASE REPORT In 1987, a 39‑year‑old Italian woman came to clinicians attention for high blood pressure and
the finding of a retroperitoneal mass (26 cm of diameter), above the right kidney, that was surgically removed. Histological examination assessed the diagnosis of pheochromocytoma [Figure 1]. After surgery, pressure values returned to normal levels. No other lesions were detected in other organs with abdominal computed tomography (CT) scan. A few months later, a CT scan demonstrated a mass in the right liver, with normal levels of urinary catecholamines and vanilmandelic acid. However, pressure values were not altered. At laparotomic exploration, the lesion was ovoidal, soft and regular, with features of a hepatic hemangioma; hence, no intervention has been realized. So, it was decided to continue only with clinical and instrumental follow‑up. In 2008, 21 years later, ultrasound revealed only the already known liver hemangioma with sharp borders, measuring 5.4 cm. In March 2012, she had recurring hypertensive crisis. Endocrinological analysis revealed that vanilmandelic acid, homovanillic acid, chromogranin A, nor‑metanephrine, metanephrine and 3‑methoxytyramine were increased. Comorbidities included: Hypothyroidism in multinodular goiter, in substitution treatment; hypertensive heart disease; aortic valve disease; chronic atrial fibrillation in oral anticoagulant treatment. Positron Emission Tomography (PET) scans relevead a single hepatic lesion of about
Journal of Cancer Research and Therapeutics - ??? - Volume ?? - Issue ?
Elena Thai, Letizia Gnetti, Annalisa Gilli1, Pietro Caruana, Raffaele Dalla Valle2, Sebastiano Buti1 Unit of Pathology, Department of Biomedical, Biotechnological and Translational Sciences (S.Bi.Bi.T.), 1 Medical Oncology Unit, Department of Emergency and General and Specialistic Medical Area, 2Clinical and Transplantation Surgery, Department of General and Specialistic Surgery, University Hospital of Parma, Parma, Italy For correspondence: Dr. Elena Thai, L'Unità Operativa, Anatomia Patologica c/o Ospedale Maggiore, Viale A. Gramsci 14, Parma-43126, Italy. E-mail: elenathai@ yahoo.com
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Thai, et al.: Very late recurrence of an apparently benign pheochromocytoma
11 cm. A CT scan showed pulmonary thromboembolism and an inhomogeneous hypodense lesion in the right liver with irregular contrast enhancement, leading to the suspect of metastasis of the pheochromocytoma resected 25 years ago. Therefore, after controlling pressure values, she underwent a right hepatectomy. During the surgical intervention, the mobilization of the right liver lobe caused important hypertensive crisis, but after the surgery, pressure values returned to normal levels. The histological examination confirmed a hepatic metastasis of pheochromocytoma: A lesion with pleomorphic cellularity, consisting of neoplastic cells arranged in nests, with finely granular cytoplasms [Figures 2 and 3], a very low mitotic index (ki67
