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Year : 2014  |  Volume : 29  |  Issue : 4  |  Page : 286-288  

Recurrent malignant pheochromocytoma with unusual omental metastasis: 68 Ga-DOTANOC PET/CT and 131 I-MIBG SPECT/CT scintigraphy findings


Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication11-Oct-2014

Correspondence Address:
Rakesh Kumar
Department of Nuclear Medicine and Positron Emission Tomography, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-3919.142654

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   Abstract 

Pheochromocytomas are rare catecholamine-secreting tumors derived from the sympathetic nervous system. The most common sites of metastasis for pheochromocytoma or extra-adrenal paraganglioma are lymph nodes, bones, lungs, and liver. Patients with known or suspected malignancy should undergo staging with computed tomography (CT) or magnetic resonance imaging as well as functional imaging (e.g. with 123 I/ 131 I-MIBG ( 131 I-metaiodobenzylguanidine) and 68 Ga-DOTANOC ( 68 Ga-labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-octreotide) positron emission tomography (PET)/CT) to determine the extent and location of disease. We present a case of recurrent malignant pheochromocytoma with unusual site of metastasis in omentum, which was positive on 68 Ga-DOTANOC PET/CT and 131 I-MIBG single-photon emission computed tomography (SPECT/)/CT scintigraphy.

Keywords: 131 I-MIBG, 68 Ga-labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-octreotide, malignant pheochromocytoma, positron emission tomography/computed tomography, Single-photon emission computed tomography/computed tomography


How to cite this article:
Arora S, Agarwal KK, Karunanithi S, Tripathi M, Kumar R. Recurrent malignant pheochromocytoma with unusual omental metastasis: 68 Ga-DOTANOC PET/CT and 131 I-MIBG SPECT/CT scintigraphy findings. Indian J Nucl Med 2014;29:286-8

How to cite this URL:
Arora S, Agarwal KK, Karunanithi S, Tripathi M, Kumar R. Recurrent malignant pheochromocytoma with unusual omental metastasis: 68 Ga-DOTANOC PET/CT and 131 I-MIBG SPECT/CT scintigraphy findings. Indian J Nucl Med [serial online] 2014 [cited 2019 Dec 13];29:286-8. Available from: http://www.ijnm.in/text.asp?2014/29/4/286/142654

A 49-year-old male who had undergone right adrenalectomy for pheochromocytoma 8-years ago presented with impaired glucose tolerance with diabetes and hypertension since 1 year. His 24 h urine vanilylmandelic acid was elevated in range of 233 mg/24 h (normal: 0-13.6 mg/24 h). His computed tomography (CT) abdomen revealed multiple intra-abdominal masses in right suprarenal, perirenal, retrocaval, peripancreatic, and in omentum with vivid arterial enhancement. The patient was referred for 68 Ga-labeled [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-NaI3-octreotide (DOTANOC) positron emission tomography (PET)/CT study for restaging purpose. 68 Ga-DOTANOC PET/CT revealed large aortocaval lymph nodal mass (6.4 × 5.3 cm) with area of necrosis and DOTANOC uptake in non-necrotic part (SUV max-3.1). Multiple peripancreatic, mesenteric, and retroperitoneal lymph nodes were noted with increased tracer uptake. It also showed multiple omental deposit with increased tracer uptake (SUVmax-2.5) [Figure 1]. 131 I-metaiodobenzylguanidine (MIBG) planer whole body scintigraphy was performed to evaluate the therapeutic potential of 131 I-MIBG in view of the metastatic nature and inoperability of the disease. 131 I-MIBG study showed increased tracer uptake in upper and mid abdomen regions. Single-photon emission computed tomography (SPECT)/CT study revealed MIBG concentrating lesions involving aortocaval, multiple peripancreatic, mesenteric and retroperitoneal lymph node, and omental deposits suggestive of recurrent disease [Figure 2]. Ultrasound-guided aspirate from right suprarenal lesion and omental lesion showed cytomorphological feature compatible with pheochromocytoma [Figure 3]. As the lesions were showing more 131 I-MIBG uptake than 68 Ga-DOTANOC uptake, the patient was taken for 131 I-MIBG therapy.
Figure 1: 68Ga-DOTANOC PET/CT revealing large aortocaval lymph nodal mass (6.4 × 5.3 cm) with area of necrosis and DOTANOC uptake in non-necrotic part (a-c, arrows). It also showed an omental deposit with increased tracer uptake (a, d, e, dotted arrows). 68Ga-DOTANOC = 68Ga-labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-octreotide, PET = Positron emission tomography, CT = Computed tomography

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Figure 2: 131I-MIBG planer whole body scintigraphy anterior (a) and posterior (b) images showing increased tracer uptake in upper and mid abdomen regions. SPECT/CT study revealed MIBG concentrating lesions involving aortocaval lymph node (c and d, arrows) and a omental deposit suggestive of recurrent disease (e and f, dotted arrows). 131I-MIBG = 131I-metaiodobenzylguanidine, SPECT = Single-photon emission computed tomography, CT = Computed tomography

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Figure 3: Ultrasound-guided aspirate from right suprarenal lesion and omental lesion (a) (×10), (b) (×20), (c) (×40) shows groups of cell in nesting pattern with abundant eosinophilic cytoplasm and rounded nuclei showing mild nuclear pleomorphism compatible with pheochromocytoma

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Pheochromocytomas are rare catecholamine-secreting tumors derived from chromaffin cells. In all, 10-50% of intra-abdominal extra-adrenal paraganglioma are malignant. [1] Metastatic spread is the only reliable criterion for the diagnosis of malignant pheochromocytoma. In 7% of the cases, metastasis occurred in more than one organ. [2],[3] The most common sites of metastasis are lungs, liver, lymph nodes, and bones. Previous studies with 111 In-Octreotide have shown higher sensitivity for detecting metastatic pheochromocytoma than for detecting benign pheochromocytoma. [4] 68 Ga-DOTANOC PET/CT showed high sensitivity for both phaeochromocytoma and paragangliomas. This is partly because of wide spectrum of affinity of 68 Ga-DOTANOC for SSTR subtypes. On the other hand, uptake of 131 I-MIBG is dependent on the expression of vesicular monoamine transporters (VMAT 1, 2). Expression of VMAT is high in benign phaeochromocytoma but is reduced in malignant phaeochromocytoma and paragangliomas. [5] In our case study, 131 I-MIBG scintigraphy and 68 Ga-DOTANOC PET/CT both showed increased tracer uptake in metastatic pheochromocytoma. This case demonstrates unusual site of omental metastasis in malignant pheochromocytoma. Occurrence of the extra-adrenal paragangliomas outside the normal distribution of the paraganglionic tissue can probably be explained by the migratory property of the neural crest cells during embryogenesis. [6],[7] These cells can form collection of paraganglionic tissue and give rise to paragangliomas. The recurrent lesions in our case were positive both on 131 I-MIBG scintigraphy and 68 Ga-DOTANOC PET/CT. But 131 I-MIBG was strongly positive, which altered the patient management, and the patient was taken for 131 I MIBG therapy.

 
   References Top

1.
Edstrom Elder E, Hjelm Skog AL, Hoog A, Hamberger A. The management of benign and malignant pheochromocytoma and abdominal paragangliomas. Eur J Surg Oncol 2003;29:278-83.  Back to cited text no. 1
    
2.
Sunaga Y, Taniguchi N, Hayashi K, Koito M, Inada M, Oyaizu N, et al. An autopsy case of extra-adrenal malignant pheochromocytoma. Gan No Rinsho 1990;36:2092-9.  Back to cited text no. 2
    
3.
Kölby L, Bernhardt P, Johanson V, Wängberg B, Muth A, Jansson S, et al. Can quantification of VMAT and SSTR expression be helpful for planning radionuclide therapy of malignant pheochromocytomas? Ann N Y Acad Sci 2006;1073:491-7.  Back to cited text no. 3
    
4.
Kaltsas G, Korbonits M, Heintz E, Mukherjee JJ, Jenkins PJ, Chew SL, et al. Comparison of somatostatin analog and meta-iodobenzylguanidine radionuclides in the diagnosis and localization of advanced neuroendocrine tumors. J Clin Endocrinol Metab 2001;86:895-902.  Back to cited text no. 4
    
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Naswa N, Sharma P, Nazar AH, Agarwal KK, Kumar R, Ammini AC, et al. Prospective evaluation of 68 Ga-DOTA-NOC PET-CT in phaeochromocytoma and paraganglioma: Preliminary results from a single centre study. Eur Radiol 2012;22:710-9.  Back to cited text no. 5
    
6.
Jaffer S, Harpaz N. Mesenteric paraganglioma: A case report and review of the literature. Arch Pathol Lab Med 2002;126:362-4.  Back to cited text no. 6
    
7.
Kudoh A, Tokuhisa Y, Morita K, Hiraki S, Fukuda S, Eguchi N, et al. Mesenteric paraganglioma: Report of a case. Surg Today 2005;35:594-7.  Back to cited text no. 7
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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