A mesenteric solid tumor with unusual features in a young male: A case report
- Authors:
- Published online on: July 25, 2017 https://doi.org/10.3892/mco.2017.1344
- Pages: 355-358
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Copyright: © Ichikawa et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
Abstract
Introduction
Primary tumors arising in the mesentery have been reported as rare tumors; however, small primary tumors are being identified more easily as diagnostic imaging systems have improved. These tumors are typically mesenchymal: Paraganglioma (1,2), lipoma (3), Schwannoma (4,5), leiomyosarcoma (6), liposarcoma (7), osteosarcoma (8), gastrointestinal stromal tumors (GIST) (9), solitary fibrous tumors (SFT) (10), hemangioma (11), lymphangioma (12), smooth muscle tumors (10) and several types of cystic tumors (13). The majority of patients with primary tumors arising in the mesentery present with abdominal pain, and ultrasonography and/or abdominal computed tomography (CT) and/or magnetic resonance imaging (MRI) are performed. The most common symptoms of these mesenchymal tumors are abdominal pain, nausea, vomiting, abdominal distention and loss of appetite, depending on their location and size. In some cases, patients are asymptomatic and the tumors are identified during routine medical check-up. A definitive diagnosis may be confirmed using pathological examination. As these mesenchymal tumors are frequently difficult to be characterized as benign or malignant via imaging, it is important to accumulate evidence of cases in order to develop guidelines for surgery, particularly for small tumors. The present case reports a primary mesenteric solid tumor in a young patient, lacking typical features, not only on imaging but on pathological examination as well.
Case report
A 16-year-old Japanese male presented to the emergency room at Moriguchi Keijinkai Hospital (Osaka, Japan) with sudden onset of vomiting and severe lower-right abdominal pain. Following further vomiting, the pain subsided completely but later reappeared and was localized to the left of the navel. A CT scan of the abdomen detected an abnormal mass 2 cm in diameter with calcification at the left side of the aorta and the height of the lower end of the left kidney (Fig. 1A and B). Contrast-enhanced CT revealed that the tumor was early enhanced in the central area, but not its marginal zone (Fig. 1C). MRI identified a hypointense signal similar to muscle tissue on T1-weighted images (Fig. 1D), and a hyperintensity within the lesion compared with the marginal zone on T2-weighted images (Fig. 1E). 18F-fluorodeoxyglucose positron emission tomography revealed no abnormal uptake in the lesion (data not shown). Angiography image identified two arteries branching from the superior mesenteric artery feeding the tumor (Fig. 1F). Tumor markers and other laboratory data suggested no specific diagnosis, and no lesions were identified by endoscopic examinations.
From these findings it was determined that the tumor originated from the mesentery. CT and contrast-enhanced CT scans suggested paraganglioma, Schwannoma, leiomyoma or leiomyosarcoma. There was, however, no tumor type that fulfilled the criteria of the MRI images for this case. As there was a risk of hemorrhage due to the hypervascularity of the lesion and as the possibility of malignancy was not able to be ruled out, laparoscopic excision of the tumor was performed following a preoperative suspected diagnosis of hemangioma.
During surgery, as the tumor was not located by endoscopy, an abdominal incision was made to investigate further. A hypervascular tumor, 3 cm in diameter, was identified in the mesentery of jejunum at the anal side of the ligament of Treitz (Fig. 2A). The tumor had no capsule but was relatively circumscribed, and was easily removed along with surrounding adipose tissue. Certain lymph nodes around the tumor were also dissected. The frozen probe from the lymph nodes contained no atypical cells. The cut surface of the tumor was gray-white and yellow, surrounded by adipose tissue (Fig. 2B).
Histologically, the hematoxylin and eosin-stained tissue contained several components. The core of this lesion was a proliferation of spindle cells with cigar-shaped or tapering nuclei and strikingly elongated brightly eosinophilic cytoplasmic processes (Fig. 3A). These cells were distributed in a copious myxoid matrix, which contained scattered lymphocytes. The calcification that was observed on CT imaging was not observed in the tissue preparation.
Immunohistochemical examination revealed that the spindle tumor cells were positive for smooth muscle actin (Fig. 3B), while negative for S-100 protein and anaplastic lymphoma kinase (data not shown). Numerous vessels and some nerve and lymphoid tissues were observed around the tumor (Fig. 3C and D). There was no significant atypia and mitotic figures were rare. From these histological findings, it was determined that the neoplastic cells were very likely to be of smooth muscle cell origin. However, a myxoid matrix with several vessels is not characteristic of leiomyoma or hemangioma. Following several consultations with pathologists experienced in soft tissue lesions, the tumor was labeled as ‘myxoid smooth muscle neoplasm of uncertain biological potential’.
The patient has been well without any additional therapy, showing no evidence of recurrence for >2 years. Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Discussion
Several types of mesenchymal tumors of mesenteric origin have been reported, including not only benign tumors, such as paraganglioma (1,2), lipoma (3) and Schwannoma (4,5), but also malignant lesions, such as leiomyosarcoma (6), liposarcoma (7) and osteosarcoma (8). In addition, there are certain mesenchymal tumors with high risk and poor prognosis, including GIST (9), solitary fibrous tumor (10), hemangioma (11), smooth muscle tumors (10), desmoid tumors (14) and fibromatosis (15). Neurofibromas in the mesentery have been reported, as in cases with von Recklinghausen's disease (16). Lymphomas and Castleman's disease must be also considered (17). In addition, other rare cases have been reported, including lymphangiomyomatosis (18), aggressive angiomyxoma (19) and non-neoplastic lesions, such as hamartoma (20), panniculitis (21), hematoma (22) and actinomycosis (23). As the ages of the patients vary, it is challenging to reach a diagnosis for such lesions according to the patient's age. Furthermore, as numerous tumors are difficult to diagnose preoperatively, pathological diagnosis of resected tumors is important for determining how to follow up the patient.
The previous case most similar to the present case was of a primary mesenteric smooth muscle tumor considered likely to behave in a malignant manner due to its large size of 20 cm in diameter (10). It is established that extrauterine smooth muscle tumors may exhibit a malignant behavior compared with uterine tumors, even if they do not exhibit aggressive histopathological characteristics. Although in our case the tumor was small, it was atypical; thus, a careful follow-up is required.
Alleviating symptoms is one of the goals of surgery, and in the current case the abdominal pain was relieved following surgery. The involvement of some nerve fibers in the proliferation of smooth muscle cells may have led to the wandering pain that was the chief complaint of the patient. The performed surgery was able to alleviate the pain experienced by the patient, in addition to preventing recurrence and hemorrhaging of the tumor.
In conclusion, the improvement of detection techniques is increasing the frequency of identification of small tumors in locations deeper within the body, such as the mesentery. Although the malignant potential in the present case remains unclear, we recommend that tumors with uncertain imaging diagnoses be resected and followed up closely, particularly in young patients, as there are numerous tumor types that are malignant or have malignant potential. It is necessary to accumulate pathological characteristics of cases with uncertain imaging diagnoses, until sufficient evidence is gathered to determine whether they are benign or malignant. The present case may contribute to considering the treatment of mesenteric tumors as one type of unclassified neoplasm.
Acknowledgements
The authors would like to thank Professor Christopher D.M. Fletcher, Department of Pathology, Harvard Medical School (Boston, USA), and Dr. Eiichi Konishi, Associate Professor, Department of Pathology, Kyoto Prefectural University of Medicine (Kyoto, Japan), for the diagnosis of the tumor.
Glossary
Abbreviations
Abbreviations:
CT |
computed tomography |
MRI |
magnetic resonance imaging |
GIST |
gastrointestinal stromal tumors |
SFT |
solitary fibrous tumors |
H-E |
hematoxylin-eosin |
References
Fujita T, Kamiya K, Takahashi Y, Miyazaki S, Iino I, Kikuchi H, Hiramatsu Y, Ohta M, Baba S and Konno H: Mesenteric paraganglioma: Report of a case. World J Gastrointest Surg. 5:62–67. 2013. View Article : Google Scholar : PubMed/NCBI | |
Ozkan Z, San Ozdemir C, Yasar G, Altas O, Koc M, Gul Y and Durdag E: An unusual mesenteric tumor ‘paraganglioma’: A case report. Iran Red Crescent Med J. 16:e168372014. View Article : Google Scholar : PubMed/NCBI | |
Ozel SK, Apak S, Ozercan IH and Kazez A: Giant mesenteric lipoma as a rare cause of ileus in a child: Report of a case. Surg Today. 34:470–472. 2004. View Article : Google Scholar : PubMed/NCBI | |
Kilicoglu B, Kismet K, Gollu A, Sabuncuoglu MZ, Akkus MA, Serin-Kilicoglu S and Ustun H: Case report: Mesenteric schwannoma. Adv Ther. 23:696–700. 2006. View Article : Google Scholar : PubMed/NCBI | |
Minami S, Okada K, Matsuo M, Hayashi T and Kanematsu T: Benign mesenteric schwannoma. J Gastrointest Surg. 9:1006–1008. 2005. View Article : Google Scholar : PubMed/NCBI | |
Sidhic AK, Ranjith M, Ali KP and Tej PR: Leiomyosarcoma of the mesentry, a rare mesentric tumour. Int J Surg Case Rep. 7C:58–60. 2015. View Article : Google Scholar : PubMed/NCBI | |
Sachidananda S, Krishnan A, Ramesh R and Kuppurao S: Primary multiple mesenteric liposarcoma of the transverse mesocolon. Ann Coloproctol. 29:123–135. 2013. View Article : Google Scholar : PubMed/NCBI | |
Heukamp LC, Knoblich A, Rausch E, Friedrichs N, Schildhaus HU, Kahl P, Tismer R, Schneider B, Büttner R and Houshdaran F: Extraosseous osteosarcoma arising from the small intestinal mesentery. Pathol Res Pract. 203:473–477. 2007. View Article : Google Scholar : PubMed/NCBI | |
Gorospe L, Simón MJ, Lima F, Esteban I, Madrid C and Hitos E: Primary mesenteric tumor with phenotypical features of gastrointestinal stromal tumors. Eur Radiol. 12 Suppl 3:S82–S85. 2002.PubMed/NCBI | |
Kalogiannidis I, Stavrakis T, Amplianitis I, Grammenou S, Mavromatidis G and Rousso D: Primary mesenteric smooth muscle tumor: An entity with unpredictable biologic behavior. Case Rep Obstet Gynecol. 2013:4836892013.PubMed/NCBI | |
Yang GZ, Li J and Jin H: Giant mesenteric hemangioma of cavernous and venous mixed type: A rare case report. BMC Surg. 13:502013. View Article : Google Scholar : PubMed/NCBI | |
Chang SC, Tu SH, Shi MY, Huang SH and Chen KM: Mesenteric lymphangioma causing bowel obstruction: Report of one case. Acta Paediatr Taiwan. 43:43–45. 2002.PubMed/NCBI | |
Liu Y, Peng Y, Li J, Zeng J, Sun G and Gao P: MSCT manifestations with pathologic correlation of abdominal gastrointestinal tract and mesenteric tumor and tumor-like lesions in children: A single center experience. Eur J Radiol. 75:293–300. 2010. View Article : Google Scholar : PubMed/NCBI | |
Ezumi K, Yamamoto H, Takemasa I, Nomura M, Ikeda M, Sekimoto M and Monden M: Dacarbazine-Doxorubicin therapy ameliorated an extremely aggressive mesenteric desmoid tumor associated with familial adenomatous polyposis: Report of a case. Jpn J Clin Oncol. 38:222–226. 2008. View Article : Google Scholar : PubMed/NCBI | |
McCormack D, Kesha K, Tittle SL and Saldinger PF: Mesenteric fibromatosis mimicking a gastrointestinal stromal tumor. Conn Med. 74:197–200. 2010.PubMed/NCBI | |
Park J: Mesenteric plexiform neurofibroma in an 11-year-old boy with von Recklinghausen disease. J Pediatr Surg. 42:E15–E18. 2007. View Article : Google Scholar : PubMed/NCBI | |
Papaziogas B, Chatzimavroudis G, Koutelidakis I, Grigoriou M and Atmatzidis K: A rare form of isolated mesenteric Castleman's disease presenting as an abdominal mass (isolated mesenteric Castleman's disease). J Gastrointestin Liver Dis. 15:171–174. 2006.PubMed/NCBI | |
Mboyo A, Flurin V, Foulet-Roge A, Bah G, Orain I and Weil D: Conservative treatment of a mesenteric lymphangiomyomatosis in an 11-year-old girl with a long follow-up period. J Pediatr Surg. 39:1586–1589. 2004. View Article : Google Scholar : PubMed/NCBI | |
Kawachi J, Isogai N, Shimoyama R, Miyake K, Ogino H and Watanabe KA: Case of Mesentric Aggressive Angiomyxoma. J Jpn Surg Assoc. 75:1607–1610. 2014. View Article : Google Scholar | |
Nagae I, Hamasaki Y, Tsuchida A, Tanabe Y, Takahashi S, Minato S, Takada K, Koyanagi Y and Aoki T: Primary omental-mesenteric myxoid hamartoma of the mesoappendix incidentally detected after abdominal trauma in a child: Report of a case. Surg Today. 35:792–795. 2005. View Article : Google Scholar : PubMed/NCBI | |
Wen YK and Chen ML: Mesenteric panniculitis: An unusual cause of dyspepsia in a hemodialysis patient. Clin Nephrol. 71:224–227. 2009. View Article : Google Scholar : PubMed/NCBI | |
Vaiphei K, Gupta V and Rajesh LS: Mesenteric hematoma mimicking a neoplasm-a case report. Ann Diagn Pathol. 10:107–109. 2006. View Article : Google Scholar : PubMed/NCBI | |
Chan YL, Cheng CS and Ng PW: Mesenteric actinomycosis. Abdom Imaging. 18:286–287. 1993. View Article : Google Scholar : PubMed/NCBI |