Abstract
Background/Aim: Carcinoma of unknown primary (CUP) poses a formidable diagnostic challenge, characterised by high mortality rates and an elusive primary tumour site. While Positron emission tomography (PET) scans are routinely employed in the initial evaluation of CUP patients, identifying the primary tumour remains an ongoing struggle. In light of this, the aim of this case report is to introduce a novel radiological description, termed the ‘Starburst’ sign, derived from distinctive PET scan appearances associated with CUP. Case Report: In this report, we present the case of a 47-year-old female patient who presented with abdominal symptoms. Upon investigation, extensive peritoneal disease was observed, yet the primary tumour source remained unidentified. Despite further diagnostic efforts, including a normal gastroscopy, a PET scan was able to confirm the presence of high-volume metastatic disease, without an identifiable primary tumour. Palliative treatment was initiated, but unfortunately, the patient’s condition deteriorated rapidly, leading to her demise. Conclusion: The ‘Starburst’ sign, a unique radiological description of CUP in PET scans, has significant potential in advancing our understanding of the disease. It provides a visual analogy to a dying star, aiding comprehension of complex pathophysiology and implications of metastatic lesions. The introduction of the ‘Starburst’ sign benefits patients and healthcare professionals, enhancing education, assessment, and treatment of CUP. This novel description contributes to knowledge in the field and can impact clinical management.
Each year in Australia, over 2,500 new cases of patients are diagnosed with carcinoma of unknown primary (CUP) (1). Unfortunately, these patients have a high mortality rate and poor prognosis. Positron emission tomography (PET) scans are usually done as part of the initial evaluation of these patients, but they rarely identify the primary tumour. We suggest that this gives a particularly distinct appearance on the PET scan that resembles a ‘burst’ or ‘dying’ star, hence, we here refer to it as the ‘Starburst’ sign (coined by the first author).
Unfortunately, while CUP is a relatively common clinical entity, PET scans can only identify a primary site in about 40% of patients (2, 3). Reasons for this may include that the primary tumour is too small to be observed, or hidden by the overwhelming metastatic burden of disease, or the patient’s immune system has attacked and destroyed the primary tumour, leaving only the secondary or metastatic disease visible on imaging.
Case Report
A 47-year-old female presented to a regional hospital with vomiting and vague diffuse abdominal pain. Initial investigations with a computed tomography (CT) scan revealed extensive peritoneal disease with no identifiable primary tumour. Upon transfer to a peritonectomy unit, further evaluation with a gastroscopy (which was normal) and a positron emission tomography (PET) scan (Figure 1) revealed that she had high volume disease. She underwent a palliative procedure (laparotomy and venting gastrostomy) and died three weeks later.
Positron emission tomography scan of a 47-year-old female with adenocarcinoma of unknown origin with the typical ‘Starburst’ sign appearance.
Discussion
CUP is a complex disease with not fully understood underlying pathophysiology. Three hypothetical scenarios have been suggested: a cancer may develop without a premalignant lesion or primary cancer; or a very early primary cancer develops that quickly metastasises, or that chromosomal instability may play a role in the aggressive presentation of metastatic disease leading to subsequent chemoresistance (4). Providing patients and trainee physicians with a simple description of this process would help them to understand their disease and the subsequent assessment and treatment process.
When a star reaches the end of its life cycle, the depletion of its core fuel initiates a sequence of events leading to its contraction and collapse due to gravitational forces. This process typically unfolds in two distinct phases. Initially, the inner core undergoes a reduction in size, followed by the inward compression of the outer core, resulting in its ultimate collapse. As a consequence of this cataclysmic event, an intense release of energy and matter occurs, manifesting in an explosive phenomenon (5). Given the striking resemblance of the observed PET scan appearance (where no primary is identifiable, but high-volume metastatic disease is present) to this celestial occurrence, the lead author aptly coined it the ‘Starburst’ sign. In this analogy, the primary tumour assumes the role of a dying star, wherein its physical remnants are diminished, and only the radiant burst-like elements of the star persist, mirroring the metastatic burden evident in PET scans. This metaphorical description encapsulates the essence of the observed radiological presentation, contributing to a deeper understanding of the pathophysiology and implications of the identified metastatic lesions.
Conclusion
To the best of our knowledge, the above description of the radiological appearance of CUP in PET scan images as a ‘Starburst’ has never been presented to the scientific community. We believe this novel description could help to better understand the complex pathophysiology of this disease process and help newly diagnosed patients and prospective health professionals in their learning.
Footnotes
Authors’ Contributions
Dr. Adam T. Cristaudo was responsible for all aspects of this submission, including the originality of the concept highlighted by the image, uniqueness, and learning points. Writing of the manuscript was carried out in collaboration with the other listed Authors Prof. David Morris and Dr. Shoma Barat. All Authors approved the final version of the manuscript.
Conflicts of Interest
The Authors have no conflicts of interest to declare in relation to this study.
- Received June 8, 2023.
- Revision received July 3, 2023.
- Accepted July 4, 2023.
- Copyright © 2023 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.
This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY-NC-ND) 4.0 international license (https://creativecommons.org/licenses/by-nc-nd/4.0).