CASE 12446 Published on 25.08.2015

Neuroendocrine cervical carcinoma – a rare tumour with agressive behaviour

Section

Genital (female) imaging

Case Type

Clinical Cases

Authors

Cristina Maciel1, 2, Óran Roche2, Giovanni Coniglio2, Anju Sahdev2

(1) Hospital São João,
Serviço de Radiologia;
Alameda Prof. Hernâni Monteiro
4200-319 Porto, Portugal;
Email:tina_maciel@yahoo.com
(2) St Bartholomew's Hospital,
Department of Radiology;
West Smithfield
London EC1A 7BE, UK
Patient

47 years, female

Categories
Area of Interest Genital / Reproductive system female ; Imaging Technique PET, MR, MR-Diffusion/Perfusion, PET-CT
Clinical History
A 49-year-old woman presented with lower abdominal and suprapubic pain with profuse vaginal discharge. At colposcopy, a large mass protruding from the external os was seen and cervical biopsies were obtained. A pelvic MRI was performed with unusual MRI findings. A supplementary FDG-PET/CT scan was performed for staging.
Imaging Findings
The pelvic MRI (Fig. 1) revealed a bulky tumour involving the whole cervical stroma, both vaginal fornices and uterine myometrium. Bilateral parametrial invasion, external iliac chain lymphadenopathy and a small volume of free fluid within the pouch of Douglas were seen. The ovaries, pelvic side walls and both kidneys were normal with no evidence of hydronephrosis.

A 18F-FDG PET/CT scan (Fig. 2, 3) demonstrated multiple metabolically active lesions in the brain (Fig. 2), lungs (Fig. 3a), left adrenal gland (Fig. 3c), peritoneum (Fig. 3d), bone, soft tissue and the vulva. Extensive lymphadenopathy with high FDG uptake was present in the pelvis (external iliac chain bilaterally) supraclavicular, subcarinal, hilar, retrocrural (Fig. 3c), aortocaval (Fig. 3d), paraaortic (Fig. 3b, d) and inguinal distributions. Intense FDG uptake was seen within the cervix and uterus corresponding to the mass seen on MRI.
Discussion
Cervical cancer consists of several histological subtypes. Neuroendocrine tumours represent 1% of cervical malignancies, comprising four categories: small cell neuroendocrine carcinoma (SCNEC), large cell neuroendocrine carcinoma (LCNEC), typical and atypical carcinoid tumours. SCNEC is the most common, accounting for approximately 80% of the cases. LCNEC, although histologically distinct, shares the same natural history as SCNEC and is treated similarly [1]. Poorly differentiated, or high grade neuroendocrine carcinomas (NEC) occur in SCNEC and LCNEC, and are the most aggressive phenotypes with a poor prognosis [2].
Cervical NEC have an aggressive clinical behaviour characterised by frequent parametrial involvement, higher frequency of lymph node metastases and early haematogenous dissemination. The most commonly involved organs include the liver, lung, adrenals, bone, bone marrow and brain [3, 4]. Brain metastases from cervical squamous cell carcinoma (SCC) and adenocarcinomas are extremely rare at presentation. However, the rate of brain metastasis from cervical NEC is uniquely high, affecting 33% of patients in a retrospective study of 15 cases [5]. The median age at diagnosis in cervical NEC is the 5th decade, in contrast to SCC and adenocarcinomas which are most common in the 2nd and 3rd decades [3].
The imaging appearances of most malignant tumours of the cervix overlap and the histological subtypes cannot be distinguished on imaging alone [6]. Neuroendocrine tumours have a non-specific morphology on MRI with scarce literature on the imaging appearances [7, 8].
Based only on the clinical presentation, with profuse vaginal discharge and suprapubic pain, cervicitis was initially included in our differential diagnosis.
PET/CT upstaged the carcinoma from FIGO stage IIB to IVB as distant metastatic disease was demonstrated.
In our case, the extent and distribution of metastatic disease raised the possibility of cervical cancer and an aggressive subtype and neuroendocrine differentiation was considered.

Colposcopic biopsy with immunohistochemical analysis confirmed a poorly differentiated cervical carcinoma with neuroendocrine differentiation. The patient received palliative chemotherapy.

This case illustrates a rare histological type of cervical cancer, with an extremely aggressive behaviour. The extensive metastatic disease (including peritoneal, lymphatic and haematogeneous dissemination) was exquisitely depicted by whole body FDG-PET/CT. In addition to advanced SCC/adenocarcinomas, the presence of aggressive features such as large invasive tumour, parametrial invasion, extensive pelvic lymphadenopathy, early distant metastasis (particularly cerebral metastasis) should raise suspicion of an aggressive histological subtype including NEC or neuroendocrine differentiation. When these features are present on pelvic MRI, a whole body PET CT should be performed given the high incidence of distant metastasis, relevant for future management [8].
Differential Diagnosis List
Neuroendocrine cervical carcinoma, with diffuse metastatic disease at presentation.
Acute cervicitis
Cervical squamous cell carcinoma
Cervical adenocarcinoma
Cervical sarcoma
Cervical lymphoma
Final Diagnosis
Neuroendocrine cervical carcinoma, with diffuse metastatic disease at presentation.
Case information
URL: https://www.eurorad.org/case/12446
DOI: 10.1594/EURORAD/CASE.12446
ISSN: 1563-4086
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