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Year : 2017  |  Volume : 22  |  Issue : 4  |  Page : 242-244

Recurrent malignant pheochromocytoma with lymph nodal metastasis in a child: A rare case

1 Department of Urology, JIPMER, Puducherry, India
2 Department of Pathology, JIPMER, Puducherry, India

Date of Web Publication12-Sep-2017

Correspondence Address:
Ramanitharan Manikandan
Associate Professor, Department of Urology, JIPMER, Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.214454

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Malignant pheochromocytoma in children manifesting as local recurrence with multiple lymph nodal metastasis is a rare entity. We report a case of a 14-year-old child with recurrent sporadic malignant pheochromocytoma presenting 8 years after primary surgery with retroperitoneal lymphadenopathy. The child underwent excision of the tumor along with retroperitoneal lymphadenectomy. Histopathology confirmed pheochromocytoma with extensive lymph node metastasis. The patient has no signs of disease recurrence till date. This report supports the long-term follow-up and aggressive surgical approach to remove all foci of tumor in recurrent pheochromocytoma.

Keywords: Malignant, pediatric, pheochromocytoma, recurrence

How to cite this article:
Mittal J, Manikandan R, Dorairajan LN, Toi PC. Recurrent malignant pheochromocytoma with lymph nodal metastasis in a child: A rare case. J Indian Assoc Pediatr Surg 2017;22:242-4

How to cite this URL:
Mittal J, Manikandan R, Dorairajan LN, Toi PC. Recurrent malignant pheochromocytoma with lymph nodal metastasis in a child: A rare case. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2021 Sep 18];22:242-4. Available from: https://www.jiaps.com/text.asp?2017/22/4/242/214454

   Introduction Top

Pheochromocytoma in children is rare with an incidence of 2/million.[1] The incidence of pediatric malignant pheochromocytoma is reported to be 10–50% of all pediatric pheochromocytomas.[2] There are only a few reports in children on the successful surgical management of malignant pheochromocytoma with lymph node metastases in the primary setting.[3] Furthermore, the treatment of such recurrent disease in the adrenal bed and in para-aortic lymph nodes is a difficult proposition. We report a rare case of a 14-year-old child with recurrent sporadic malignant pheochromocytoma who presented 8 years after left adrenalectomy for pheochromocytoma.

   Case Report Top

A 14-year-old child who underwent left adrenalectomy for pheochromocytoma originating in the left adrenal gland at the age of 6 years, presented to the emergency medical service with accelerated hypertension and blurring of vision. Ultrasonogram and magnetic resonance imaging showed a 4 cm × 3 cm recurrence in the left adrenal fossa with multiple para-aortic lymph nodes [Figure 1].131 I-MIBG showed avid uptake at the same sites. The plasma-free normetanephrine was elevated (567 pg/ml) and serum cortisol was within normal limits. The fundus examination showed grade 4 hypertensive retinopathy. The primary histology was reported as having classical Zellballen pattern suggestive of pheochromocytoma without malignant features. Familial syndromes were ruled out. The preoperative preparation was performed with alpha adrenergic receptor blocker (prazosin 5 mg BD) followed by beta-adrenergic receptor blocker (propanol 20 mg BD) with high salt diet and liberal water intake. Laparotomy under general anesthesia was performed, and the tumor was excised along with retroperitoneal lymphadenectomy. The blood pressure of the child normalized on the 3rd postoperative day. The histopathology was suggestive of pheochromocytoma with extensive lymph node metastasis indicating malignant features [Figure 2]. The patient is now free of antihypertenive medications. Follow-up protocol included metabolic testing at 3 months and 1 year and computed tomography (CT) scan imaging at the end of 1st year. There is no evidence of disease recurrence in the child till date.
Figure 1: Coronal T2 Trufi magnetic resonance images of the abdomen show multiple lobulated T2 heterogeneous hyperintense lesions in the left adrenal bed and paravertebral region, closely abutting the medial border of the left kidney suggestive of recurrence in the left adrenal bed with para-aortic lymphadenopathy

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Figure 2: Section shows tumor cells arranged in alveolar pattern surrounded by capillary-rich framework giving a characteristic Zellballen pattern. The periphery shows lymphoid tissue (H and E, ×40) (a). The cytoplasm is amphiphilic, granular, with round to ovoid nuclei, coarsely clumped chromatin, and prominent nucleoli (H and E, ×40) (b)

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   Discussion Top

Malignant pheochromocytoma is defined as an occurrence of distant metastasis in nonchromaffin tissue and not by local invasion.[4] The incidence of malignancy among pediatric patients is higher as compared to adult population (47% vs. 10%–30%).[2] The typical sites of metastasis include bone, lung, liver, and lymph nodes. The risk factors for pediatric malignant pheochromocytoma include the extra-adrenal location, primary tumor >6 cm, sporadic nature, gland weight >250 g, and early onset postoperative hypertension.[2] The risk factors for recurrence are predominantly due to rupture of tumor capsule or incomplete tumor resection. The reported incidence of local tumor recurrence is 16%–26% after negative margins for primary surgery.[2],[3] The incidence of recurrence following adrenal conserving surgery for the pheochromocytoma is reported to be 15%–20% and is almost similar to total adrenalectomy.[5] Positron emission tomography-CT with different radiopharmaceuticals has a significant role in the detection of metastasis when MIBG scintigraphy is negative.[6]

Surgical resection is the mainstay of treatment due to poor response to medical management. The role of laparoscopic surgery in this setting is limited to specialized centers with experienced laparoscopic surgeons. The overall 5-year and 10-year disease-specific survival rate for malignant pediatric pheochromocytoma is reported to be 78% and 31%, respectively.[2] The outcome of children with recurrent malignant pheochromocytoma is variable, with isolated reports of patients surviving more than two decades.[7] The possible explanation for the high survival rate among children may be due to tumor detection at an earlier age, less biologically aggressive tumor, and aggressive surgical management.[2] In cases of unresectable disease, surgical debulking and local therapies such as ablation or radiotherapy can provide effective palliation.[2],[8] Radiolabelled MIBG (I 131) or somatostatin analogs, separate or in synergistic combination, may achieve tumor volume control and partial hormonal response in 50% of the patients with metastatic pheochromocytoma.[9] The chemotherapeutic drugs have shown poor response rate in this setting.[2],[9] The role of molecular targeted therapies such as m-TOR inhibitors, tyrosine kinase inhibitors, HER-2/neu inhibitors, and antiangiogenic agents are currently in trial setting only.[9]

Repeat metabolic testing should be performed 2–6 weeks after surgery. These patients require long-term annual biochemical follow-up. Subsequent imaging or MIBG scan is indicated only in the setting of elevated biochemical parameters.[10]

The occurrence of metastatic pheochromocytoma in lymph nodal tissue is extremely rare, and this makes our case unique. Although there are reports of lymph nodal recurrence in adult pheochromocytoma, to the best of our knowledge, it has not been documented in children.

In malignant pheochromocytoma, careful sustained clinical and biochemical follow-up is advocated as tumor recurrence may occur years after the initial surgery. In such children even with lymph nodal metastasis, aggressive surgical approach should be considered because it offers effective clinical and functional control of the disease as seen in our patient.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Ein SH, Shandling B, Wesson D, Filler RM. Recurrent pheochromocytomas in children. J Pediatr Surg 1990;25:1063-5.  Back to cited text no. 1
Pham TH, Moir C, Thompson GB, Zarroug AE, Hamner CE, Farley D, et al. Pheochromocytoma and paraganglioma in children: A review of medical and surgical management at a tertiary care center. Pediatrics 2006;118:1109-17.  Back to cited text no. 2
Ciftci AO, Tanyel FC, Senocak ME, Büyükpamukçu N. Pheochromocytoma in children. J Pediatr Surg 2001;36:447-52.  Back to cited text no. 3
Thompson LD, Young WF Jr, Kawashima A, Komminoth P, Tischler AS. Malignant adrenal pheochromocytoma. In: De Lellis RA, Lloyd RV, Heitz PU, Eng C, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Endocrine Organs. IARC Press Edition: Lyon; 2004; p. 147-50.  Back to cited text no. 4
Lee JE, Curley SA, Gagel RF, Evans DB, Hickey RC. Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma. Surgery 1996;120:1064-70.  Back to cited text no. 5
Yun M, Kim W, Alnafisi N, Lacorte L, Jang S, Alavi A. 18F-FDG PET in characterizing adrenal lesions detected on CT or MRI. J Nucl Med 2001;42:1795-9.  Back to cited text no. 6
Luiz HV, da Silva TN, Pereira BD, Santos JG, Gonçalves D, Manita I, et al. Malignant paraganglioma presenting with hemorrhagic stroke in a child. Pediatrics 2013;132:e1709-14.  Back to cited text no. 7
Fishbein L, Bonner L, Torigian DA, Nathanson KL, Cohen DL, Pryma D, et al. External beam radiation therapy (EBRT) for patients with malignant pheochromocytoma and non-head and –Neck paraganglioma: Combination with 131I-MIBG. Horm Metab Res 2012;44:405-10.  Back to cited text no. 8
Grogan RH, Mitmaker EJ, Duh QY. Changing paradigms in the treatment of malignant pheochromocytoma. Cancer Control 2011;18:104-12.  Back to cited text no. 9
Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665-75.  Back to cited text no. 10


  [Figure 1], [Figure 2]


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