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F-FDG PET/CT in Metastatic Extramammary Paget’s Disease
Alex Cheen Hoe Khoo, MBBS, MMed, FANMB,* and Kheng Wei Yeoh, MSc, MBBS, MRCP, MPH, FRCR†

REFERENCES
Abstract: Extramammary Paget's disease (EMPD) is a rare disease with an
estimated prevalence of 0.1 to 2.4 per 1,000,000 person-years. Metastatic 1. Tian Y, Wu HB, Li DL, et al. Utility of 18F-FDG PET/CT in the diagnosis and
EMPD has a poor prognosis with a 5-year survival of approximately 7%. Local staging of extramammary Paget's disease. Nucl Med Commun. 2015;36:892–897.
therapy is the only curative option with surgery being recommended for resect- 2. Ohara K, Fujisawa Y, Yoshino K, et al. A proposal for a TNM staging system
able disease. It is therefore crucial to be able to stage such patients appropriately. for extramammary Paget disease: retrospective analysis of 301 patients with
invasive primary tumors. J Dermatol Sci. 2016;83:234–239.
The utility of 18F-FDG PET/CT for this disease is not well established. We
3. Wagner G, Sachse MM. Extramammary Paget disease—clinical appearance,
share a case on how 18F-FDG PET/CT was used to stage metastatic EMPD. pathogenesis, management. J Dtsch Dermatol Ges. 2011;9:448–454.
Key Words: extramammary, Paget's disease, metastatic, 18F-FDG, PET/CT 4. Isrow D, Oregel KZ, Cortes J, et al. Advanced extramammary Paget's disease
of the groin, penis, and scrotum. Clin Med Insights Oncol. 2014;8:87–90.
(Clin Nucl Med 2019;44: 808–809)
5. Zollo JD, Zeitouni NC. The Roswell Park Cancer Institute experience with
extramammary Paget's disease. Br J Dermatol. 2000;142:59–65.
6. Oashi K, Tsutsumida A, Namikawa K, et al. Combination chemotherapy for met-
Received for publication March 14, 2019; revision accepted June 5, 2019. astatic extramammary Paget disease. Br J Dermatol. 2014;170:1354–1357.
From the Departments of *Nuclear Medicine, and †Oncology, Penang Adventist
Hospital, George Town, Penang, Malaysia. 7. Zhu Y, Ye DW, Yao XD, et al. Clinicopathological characteristics, manage-
The manuscript has not been published before or is not under consideration for ment and outcome of metastatic penoscrotal extramammary Paget's disease.
publication anywhere else and has been approved by all co-authors. Br J Dermatol. 2009;161:577–582.
Conflicts of interest and sources of funding: none declared. 8. Li ZG, Qin XJ. Extensive invasive extramammary Paget disease evaluated by
Ethical Statement: The study was approved by an institutional review board or F-18 FDG PET/CT: a case report. Medicine. 2015;94:e371–e371.
equivalent and has been performed in accordance with the ethical standards
laid down in the 1964 Declaration of Helsinki and its later amendments.
The subject in the study gave written informed consent.
Authors' Contribution: Khoo proposed and co-wrote the manuscript. Yeoh co-
wrote the manuscript. Both reviewed the final manuscript.
Correspondence to: Alex Khoo Cheen Hoe, MBBS, MMed, FANMB, Department
of Nuclear Medicine, Penang Adventist Hospital, 456, Jalan Burma 10350
Georgetown, Penang, Malaysia. E-mail: dr.alexkhoo@gmail.com.
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ISSN: 0363-9762/19/4410–0808
DOI: 10.1097/RLU.0000000000002739

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Clinical Nuclear Medicine • Volume 44, Number 10, October 2019 Metastatic Extramammary Paget's Disease

FIGURE 1. A fit 80-year-old man with underlying diabetes mellitus and hypertension presented in mid-2015 with scrotal
itchiness and erythema. Biopsy of the scrotal lesion revealed extramammary Paget’s disease (EMPD) of the scrotum, which was
surgically excised in November 2015. Within 3 months, he had recurrent disease involving the left inguinal nodes with no
systemic involvement, confirmed on 18F-FDG PET/CT scan. This nodal recurrence was duly treated surgically, and he remained
well for 11 months before developing another locoregional recurrence. Systemic disease was again excluded with 18F-FDG
PET/CT scan before a salvage nodal surgery was performed in February 2017. A restaging 18F-FDG PET/CT scan performed
22 months later confirmed disseminated disease when he presented with yet another recurrence in the left groin region (A).
The PET MIP images revealed extensive FDG-avid metastases to the axial and appendicular skeletons, distant nodes (neck,
mediastinal, abdomen, and pelvis), and muscle. B–D, Figures show the fused axial PET/CT images of the metastatic disease
(white arrow) involving the mediastinal nodes, right ilium, and left scrotal region as well as left pectineus muscle, respectively.
The most metabolically active cutaneous lesion (at the left inguinal region; SUV, 5.5) was 1.6-cm thick, whereas the least intense
visible cutaneous lesion (at the left anterior superior iliac spine region; SUV, 2.6) was 0.6 cm. Tian et al1 demonstrated that the
thickness of the cutaneous lesion was more crucial compared with its size for positive detection on 18F-FDG PET/CT. The patient
was upstaged as stage IV based on the TNM recommendations by Ohara et al.2 On the account of these findings, systemic
chemotherapy has been initiated. Paget’s disease, which frequently affects patients over the age of 60 years, is also known as
eczematoid carcinoma due to its frequently misdiagnosed nonspecific eczematous dermal presentation.3,4 This rare
intraepithelial adenocarcinoma can be divided into mammary Paget’s disease and extramammary Paget’s disease (EMPD),
and can be associated with concurrent malignancy. The prevalence of EMPD is estimated to be 0.1 to 2.4 per 1,000,000
person-years.2 EMPD, a slow-growing cutaneous adenocarcinoma involving apocrine gland-rich sites, frequently affects the
vulva (female), penis and scrotum (male), perianal region, and rarely the axilla. The recommended treatment option for
localized primary EMPD is surgical excision. However, the recurrence rate for all sites is in the range of 35% to 44%.5 Local
ablative approaches such as radiotherapy is indicated in inoperable cases and as adjuvant therapy after tumor excision in
high-risk patients. Although uncommon, EMPD can become invasive with metastatic potential, and hence initial staging of
the tumor is essential. In the setting of metastatic EMPD, there is no established chemotherapy regimen with various options
being proposed.6 There is limited literature on the utility of 18F-FDG in the management of EMPD, especially in the detection
of metastatic disease.7,8 Cure can only be achieved in this condition when the disease is localized making the detection of
metastatic disease crucial in the optimal management of this condition.4

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