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CASE REPORT
Year : 2018  |  Volume : 17  |  Issue : 1  |  Page : 62-64

Alveolar soft part sarcoma presenting as hypervascular adrenal metastasis


Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

Date of Web Publication5-Jan-2018

Correspondence Address:
Manjunath Goroshi
Department of Endocrinology, Seth G S Medical College and KEM Hospital, Parel, Mumbai - 400 012, Maharashtra
India
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DOI: 10.4103/wjnm.WJNM_8_17

PMID: 29398970

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   Abstract 


Hypervascular adrenal masses include pheochromocytoma, metastases caused by clear renal cell carcinoma/hepatocellular carcinoma. Alveolar soft part sarcoma (ASPS) causing hypervascular metastases is not described in the literature. Here, we describe the first case of ASPS presenting as hypervascular metastasis. Our case was a 23-year-old male incidentally detected right adrenal mass during the evaluation of pain in the abdomen. On computed tomography (CT), adrenal mass showed bright enhancement in early arterial phase (unenhanced Hounsfield unit [HU]-45.3; arterial phase HU-158.2). 18- flurodeoxyglucose positron emission tomography/CT showed multiple lesions and was confirmed histologically to be due to ASPS.

Keywords: Adrenal contrast-enhanced computed tomography, hypervascular metastasis, soft tissue sarcoma, washout


How to cite this article:
Goroshi M, Lila AR, Bandgar T, Shah NS. Alveolar soft part sarcoma presenting as hypervascular adrenal metastasis. World J Nucl Med 2018;17:62-4

How to cite this URL:
Goroshi M, Lila AR, Bandgar T, Shah NS. Alveolar soft part sarcoma presenting as hypervascular adrenal metastasis. World J Nucl Med [serial online] 2018 [cited 2021 Jan 19];17:62-4. Available from: http://www.wjnm.org/text.asp?2018/17/1/62/222287




   Introduction Top


Hyper-vascular adrenal masses include pheochromocytoma, metastases caused by clear renal cell carcinoma/hepatocellular carcinoma. Alveolar soft part sarcoma causing hyper-vascular metastases is not described in the literature. Here we describe first case of alveolar soft part sarcoma presenting as hyper-vascular metastasis.


   Case Report Top


A 23-year-old male patient presented with the early satiety, pain in the abdomen, and weight loss (5–6 kg) for the past 4 months. Ultrasonography (USG) abdomen showed large right suprarenal mass (9.5 cm × 8.8 cm × 12.5 cm) for which endocrinology referral was sought. The patient underwent multiphase contrast-enhanced computed tomography (CECT) which includes unenhanced, early arterial (20 s), early venous (60 s), and delayed (15 min) phases. CECT [Figure 1] showed rounded right suprarenal mass with central areas of necrosis and had a size of 9.9 cm (maximum size on axial images). Mass showed heterogeneous contrast enhancement, and it showed bright enhancement in arterial phase (Hounsfield unit [HU]-158.2) with good washout (Absolute washout percentage – 70.1%). On biochemical evaluation, plasma metanephrines (44.1 pg/ml, normal range <180 pg/ml) and normetanephrines (30.4 pg/ml, normal range <180 pg/ml) were normal ruling out secretory pheochromocytoma. I131 metaiodobenzylguanidine scan was done to rule out the possibility of nonsecretory pheochromocytoma, which showed no significant uptake in the mass. On morning 8 am cortisol (13.85 μg/dl, normal range <30 μg/dl) and overnight dexamethasone suppression cortisol (0.8 μg/dl, normal range <1.8 μg/dl) ruled out the secretory adrenal cortical carcinoma. 18-flurodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) revealed [Figure 2] multiple hypermetabolic lesions at right adrenal mass (maximum standardized uptake value [SUVmax]: 4.52), lytic lesion in the left scapula (SUVmax: 4.46), and soft tissue mass (7.3 cm) noted in the left gluteal region eroding the left iliac bone, acetabulum, inferior pubic rami and possibly invading the hip joint (SUVmax-5.5). USG-guided biopsy from the adrenal and gluteal region was done. Both biopsy cores showed tumor cells arranged as nests separated by thin vascular septae. Cells were discohesive with central round nucleus and moderate eosinophilic cytoplasm on microscopy [Figure 3]a. On immunohistochemistry, tumor cells were positivity for TFE [Figure 3]b and epithelial membrane antigen (focal) and were negative for cytokeratin, synaptophysin, inhibin, melan-A, and PAX8 diagnostic of alveolar soft part sarcoma (ASPS). The final diagnosis was primary gluteal region ASPS with hypervascular adrenal metastasis. The patient was referred for palliative chemotherapy as the patient had distant metastases.
Figure 1: Multiphase CECT showing right adrenal mass with maximum diameter of 9.9 cm (a) unenhanced phase (HU of 45.3) with central areas of necrosis, (b) early arterial phase showing bright enhancement of mass (HU-158.2), (c) early venous phase (HU-98.6) showing washout as compared to early arterial phase, (d) 15-min delayed phase (HU-61.2) showing washout. CECT: contrast-enhanced computed tomography, HU: Hounsfield unit. Technique: 64-slice multidetector CT system (Brilliance 64, Philips Healthcare, Best, and the Netherlands) with imaging done at baseline, 20 s (early arterial), 1 min (early venous), and 15 min (delayed)

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Figure 2: (a) 18F-FDG avid lesion in the right adrenal mass (SUVmax 4.52), (b) 18F-FDG avid lesion in the left gluteal region (SUVmax-5.5), (c) 18F-FDG avid lesion in the left scapular region (SUVmax 4.6), (d and e) Coronal sections of FDG and CECT showing all three lesions (arrows), 18F-FDG: 18-flurodeoxyglucose positron emission tomography/computed tomography, SUVmax: maximum standardized uptake value, CECT: contrast-enhanced computed tomography. Technique: whole-body 18-F-FDG PET/CT was performed in a BGO plus, full ring PET/CT scanner with intravascular contrast injection

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Figure 3: (a) Hematoxylin and eosin staining under light microscopy (×40) showing tumor cells arranged as nests separated by thin vascular septae with central round nucleus and moderate eosinophilic cytoplasm, (b) on immunohistochemistry, tumor cells showed positivity for TFE suggestive of alveolar soft part sarcoma

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   Discussion Top


In recent years, adrenal masses are receiving increased attention due to surge in the incidence of adrenal incidentaloma.[1] Classification of adrenal masses depending on the etiology has become important as management varies from observation to surgical resection.[2] Moreover, it is imperative to not miss a functional/malignant lesion, due to the low prevalence of such conditions.[2] Our patient had hypervascular adrenal lesion on computed tomography (unenhanced HU-45; arterial phase HU-158). 18F-FDG PET/CT showed multiple lesions and was confirmed histologically to be due to ASPS. CECT-derived absolute and relative washout percentages were used to differentiate adenoma and nonadenoma adrenal masses. However, the hypervascular adrenal masses usually do not follow these washout patterns.[3] Hypervascular adrenal masses constitute mainly pheochromocytoma and hypervascular metastasis. Pheochromocytoma is classically characterized as brightly enhancing adrenal masses, but it can have different characteristics on washout patterns from good washout to poor washout.[4] Hypervascular adrenal metastasis may have similar washout to adenomas on delayed contrast-enhanced CT images.[3] Clear cell renal carcinoma and hepatocellular carcinoma are described in the literature as hypervascular adrenal metastasis.[3] To the best of our knowledge, our case is first to report hypervascular adrenal metastasis caused by ASPS.

ASPS most often highly vascular and can present with metastasis, particularly in cases in the extremities.[5] In our case, both primary and adrenal metastasis showed similar high vascularity. This might be the reason for bright enhancement in the early arterial phase of CECT.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Mansmann G, Lau J, Balk E, Rothberg M, Miyachi Y, Bornstein SR. The clinically inapparent adrenal mass: Update in diagnosis and management. Endocr Rev 2004;25:309-40.  Back to cited text no. 1
[PUBMED]    
2.
Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol 2016;175:G1-34.  Back to cited text no. 2
[PUBMED]    
3.
Choi YA, Kim CK, Park BK, Kim B. Evaluation of adrenal metastases from renal cell carcinoma and hepatocellular carcinoma: Use of delayed contrast-enhanced CT. Radiology 2013;266:514-20.  Back to cited text no. 3
[PUBMED]    
4.
Johnson PT, Horton KM, Fishman EK. Adrenal mass imaging with multidetector CT: Pathologic conditions, pearls, and pitfalls. Radiographics 2009;29:1333-51.  Back to cited text no. 4
[PUBMED]    
5.
Kim HS, Lee HK, Weon YC, Kim HJ. Alveolar soft-part sarcoma of the head and neck: Clinical and imaging features in five cases. AJNR Am J Neuroradiol 2005;26:1331-5.  Back to cited text no. 5
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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