|Year : 2019 | Volume
| Issue : 3 | Page : 70-72
Ulcerated duodenal gastrointestinal stromal tumor – Demonstration of an interesting radiological sign (the Torricelli-Bernoulli sign)
Pradosh Kumar Sarangi1, Nachiketa Mangaraj2, Keshaba Panigrahy3, Ankush Jajodia4
1 Department of Radiodiagnosis, SCB Medical College and Hospital, Cuttack, Odisha, India
2 Department of Radiodiagnosis, GSVM Medical College and LLR Hospital, Kanpur, Uttar Pradesh, India
3 Department of Radiodiagnosis, AIIMS, Bhubaneswar, Odisha, India
4 Department of Radiodiagnosis, Rajiv Gandhi Cancer Institute and Research Centre, Delhi, India
|Date of Web Publication||27-Dec-2019|
Dr. Pradosh Kumar Sarangi
Department of Radiodiagnosis, SCB Medical College and Hospital, Cuttack, Odisha
Source of Support: None, Conflict of Interest: None
The Torricelli-Bernoulli sign is a useful computed tomographic sign seen in ulcerated/necrotic gastrointestinal stromal tumor (GIST) or leiomyosarcoma characterized by the collection of air in the nondependent aspect of larger gastrointestinal cavitating tumors. This sign is based on the physical principle of the law of Torricelli's and Bernoulli's principle. Herein, we describe this interesting imaging sign in an adult male with malignant duodenal GIST with extensive hepatic, peritoneal, omental, and lymph nodes (though rare in GIST) metastasis. The patient succumbed few days after admission.
Keywords: Crescentic necrosis, duodenal gastrointestinal stromal tumor, Torricelli-Bernoulli sign, ulceration
|How to cite this article:|
Sarangi PK, Mangaraj N, Panigrahy K, Jajodia A. Ulcerated duodenal gastrointestinal stromal tumor – Demonstration of an interesting radiological sign (the Torricelli-Bernoulli sign). Oncol J India 2019;3:70-2
|How to cite this URL:|
Sarangi PK, Mangaraj N, Panigrahy K, Jajodia A. Ulcerated duodenal gastrointestinal stromal tumor – Demonstration of an interesting radiological sign (the Torricelli-Bernoulli sign). Oncol J India [serial online] 2019 [cited 2020 Jul 11];3:70-2. Available from: http://www.ojionline.org/text.asp?2019/3/3/70/274095
| Introduction|| |
Gastrointestinal stromal tumors (GISTs) were first described by Clark and Mazur in 1983 for smooth muscle neoplasms of the gastrointestinal tract, which are immunohistochemically different from leiomyoma, leiomyosarcomas, and neurogenic tumors. These tumors arise from interstitial cells of Cajal that express a tyrosine kinase growth factor receptor, also called c-kit protein-CD117 found in chromosome 4. Torricelli-Bernoulli sign is an useful computed tomographic sign for identifying ulcerating neoplasms originating from the gastrointestinal tract.,,,, When the intra-abdominal mass is large, the identification of organs from the origin of the mass is challenging on cross-sectional imaging. Such imaging sign when present increases the diagnostic confidence of large GIST or leiomyosarcoma.
| Case Report|| |
A 30-year-old male patient from low-scocioeconomic status presented to the outpatient department of surgery with a complaint of vague pain abdomen with increasing severity and gradual swelling for the past 5 months.
On examination, the abdomen was tense and swollen. He was advised ultrasonography of the abdomen, which revealed a large heterogeneous soft-tissue mass of size ~ 10 cm × 11 cm in the central abdomen with multiple similar lesions around it. There were multiple large hypoechoic lesions in the liver suggestive of metastasis. No conclusive diagnosis was made from the ultrasound except the malignant nature of the lesion. He was further advised for contrast-enhanced computed tomography (CECT) of the abdomen. CECT of the abdomen showed an exophytic lobulated heterogeneous enhancing soft-tissue lesion of size 9.0 cm × 7.6 cm × 10.7 cm corresponding to anteroposterior × mediolateral × craniocaudal length, respectively, arising from the third part of the duodenum with central nonenahncing area suggestive of necrosis [Figure 1]. There were air and oral contrast within the mass leading to an area of necrosis with two separate contrast-filled fistulous communications with duodenal lumen suggestive of mucosal ulceration. Collection of air in the nondependent aspect of larger cavitating tumors with necrosis is known as the “Toricelli-Bernouilli” crescentic necrosis sign, which confirms the gastrointestinal origin of the neoplasm. There were also multiple livers, mesenteric, and retroperitoneal metastases [Figure 1]. An imaging diagnosis of large duodenal malignant GIST or leiomyosarcoma was made.
|Figure 1: Contrast-enhanced computed tomography axial section showing a large exophytic heterogenously enhancing soft-tissue lesion arising from the third part of the duodenum with crescentic necrosis and air foci within (Torricelli-Bernoulli Sign) (a) and multiple hepatic metastases (b)|
Click here to view
He underwent ultrasound-guided biopsy of the mass lesion. Histopathology revealed a smooth muscle neoplasm [Figure 2]. Immunohistochemistry was positive for CD34 and c-kit (CD117); hence, confirming to be high-risk GIST [Figure 3]. He was started systemic chemotherapy with oral tablet imatinib mesylate 400 mg twice daily. The patient, however, succumbed 2 days after the admission.
|Figure 2: Histopathological examination (H and E, ×100) of tru-cut biopsy sample of duodenal mucosal mass showing (a) submucosal growth with hypocellularity (×100) and (b) spindle cells with medium density, without mitosis (H and E, ×400)|
Click here to view
|Figure 3: Photomicrograph of tumor fragments showing cytoplasmic reactivity for CD117 (immunohistochemistry, ×400)|
Click here to view
| Discussion|| |
GISTs account for 1%–3% of all gastrointestinal tumors. They originate from the interstitial cells of Cajal (pacemaker of intestine) and are frequently associated with mutations in the c-kit gene (CD 117). They can involve the gastrointestinal tract anywhere from the esophagus to anus with the stomach being the most common site (37%–70%) and esophagus being the least common site (<2%). Duodenal GIST accounts for 9% and is relatively uncommon. GIST can also occur in the mesentery, omentum, and retroperitoneum and termed as extraintestinal GIST.
On imaging, these are submucosal tumors arising from the wall of hollow viscus with exophytic or endoluminal growth. When the lesion is large, it is difficult to know origin of the tumor. Presence of a specific imaging sign, that is, Toricelli-Bernouilli sign helps in identifying gastrointestinal nature of the tumor. This sign was first described by Fortman in gastric leiomyosarcoma in 1999. It is seen in computed tomography as a stream of air bubbles arising from an ulcerating neoplasm into a fluid-filled viscus such as duodenum, as in our case., In other words, collection of air in the nondependent aspect of larger gastrointestinal cavitating tumors with necrosis produces this sign on computed tomography (CT) scan. In our case, there were air and oral contrast seen within the central necrosis of tumor due to mucosal ulceration causing communication with the duodenal lumen.
This sign is based on the two distinct physical principles of the law of Torricelli's and Bernoulli's principle acting in unison, which is the law of hydrodynamics involving fluid and gas. We are not going to describe these physical principles in detail. In short, the law of Torricelli relates the efflux velocity of liquid through the orifice in a vessel and the height of liquid above it. Bernoulli's principle states that when the friction is negligible, the flow velocity of a gas or fluid in a tubular structure is inversely related to pressure. Torricelli-Bernoulli phenomenon is initiated in the upright position when the ulcerating neoplasm is above the air-fluid level. In the recumbent position, the ulcer crater is covered by the fluid, according to the Torricelli's theory, because of the fluid influx. A noncompressible environment is created, and the fluid forces the gas out of the ulcer in accordance with Bernoulli's principle. This sign is indirect evidence of gastrointestinal origin of the mass, especially when the lesion is very large, as in our case. This sign has been described in GIST and leiomyosarcoma.,,,,,, Hence, other gastrointestinal tumor-like lymphoma, adenocarcinoma can be confidently ruled out from imaging. Sandrasegaran et al. in their study found this sign in axial CT scan in only one case (5.3%) of GIST involving the stomach out of 19 primary GISTs. This sign is rarely seen in smaller intestinal GIST (≤5 cm in diameter) and is generally seen in large tumors ≥6 cm in size where macroscopic necrosis is common. Obviously, it will be easy to identify origin of the lesion if the tumor is small. This sign has implications in larger lesions with difficult identification of origin of lesion.
| Conclusion|| |
Many radiologists are not aware of the significance of the Torricelli-Bernoulli sign. We suggest looking for this sign in cross-sectional imaging, which might increase the diagnostic confidence of GIST or leiomyosarcoma. This sign will not be demonstrated when there is no central necrosis of tumor and no mucosal ulceration as the physical principle of Torricelli and Bernoulli cannot be applied.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al.
Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998;279:577-80.
Fortman BJ. Torricelli-Bernoulli sign in an ulcerating gastric leiomyosarcoma. AJR Am J Roentgenol 1999;173:199-200.
Sureka B, Bansal K, Arora A. Torricelli-Bernoulli sign in gastrointestinal stromal tumor. AJR Am J Roentgenol 2015;205:W468.
Tajima T, Nishi T, Tomioku M, Ogimi T, Chan LF, Okazaki T, et al
. Perforated gastrointestinal stromal tumor in the small intestine: A rare case of Torricelli-Bernoulli sign. Mol Clin Oncol 2018;9:399-402.
Sureka B, Mittal MK, Mittal A, Sinha M, Thukral BB. Imaging spectrum of gastrointestinal stromal tumor. Indian J Med Paediatr Oncol 2014;35:143-8.
] [Full text]
Kurokawa S, Morikawa A, Kubo T, Morita T. Torricelli-Bernoulli sign in a large intestine gastrointestinal stromal tumor. Intern Med 2014;53:2547.
Sandrasegaran K, Rajesh A, Rushing DA, Rydberg J, Akisik FM, Henley JD, et al.
Gastrointestinal stromal tumors: CT and MRI findings. Eur Radiol 2005;15:1407-14.
Nishida T, Kumano S, Sugiura T, Ikushima H, Nishikawa K, Ito T, et al.
Multidetector CT of high-risk patients with occult gastrointestinal stromal tumors. AJR Am J Roentgenol 2003;180:185-9.
[Figure 1], [Figure 2], [Figure 3]