대한흉부영상의학회 Weekly Chest Cases

Case No : 575 Date 2008-11-03

Courtesy of Soon Mo Park, MD, Joo Sung Sun, MD, Kyung Joo Park, MD. / Ajou University School of Medicine, Radiology, Korea
Age/Sex 16 / F
Chief ComplaintRecurrent abdominal pain, chronic dysphagia

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Diagnosis With Brief Discussion

Diagnosis: Diffuse esophageal and anorectal leiomyomatosis
Radiologic Findings: A posteroanterior chest radiograph revealed huge abnormal convex shadow and show soft tissue lobulated opacities, mediastinal widening, and deviation or displacement of the paraesophageal stripes.

Contrast-enhanced computed tomography of the chest and abdomen demonstrated bulky homogenous low density mass at posterior mediastinum and circumferential low density mass in lower rectum, same character with esophageal mass. The mass extended at the level of the supraaortic arch and encase the stomach carina and esophagus. The trachea and main bronchi were not disturbed and the lungs were clear. The esophageal and rectal masses were low density to skeletal muscle and did not show significant intravenous contrast enhancement.
Brief Review: Smooth muscle tumors comprise approximately 1% of all tumors of the gastrointestinal tract [1]; 66% occur in the stomach, 24% in the small bowel, 7% in the esophagus and 2% in the colon [2]. Multiple lesions are rare, occurring in less than 2.4% of cases.

Leiomyomata are the most common benign neoplasms of the esophagus but are still infrequent in comparison to malignant tumors of this organ [3]. Twenty-five cases have been described in children [4]. Esophageal leiomyoma are most commonly found in the middle and distal thirds of the esophagus, because the muscularis propria is composed of smooth muscle in this portion of the esophagus. Although solitary tumors are more common in older individuals and present as discrete ovoid masses, the rare multifocal leiomyomas and diffuse leiomyomatosis are more likely to be seen in children and younger adults [5]. Anorectal leiomyomatosis is exceedingly rare in the pediatric population, having previously been documented in only a few cases [6, 7, 8]. There is a recognized association between esophageal leiomyomatosis and Alport's syndrome (hematuric nephritis, neurosensorial deafness, congenital cataracts) [9, 10]. Even more rare is a synchronous or metachronous association with anorectal leiomyomatosis, with such cases reported [6, 7, 8].
References: 1. Golden T, Stout AP. Smooth muscle tumours of the gastrointestinal tract and retroperitoneal tissues. Surgical Gynaecology and Obstetrics 1941;73:784-810
2. Baker HL, Good CA. Smooth muscle tumours of the alimentary tract. American Journal of Roentgenology 1955;74:246-255.
3. Seremetis MG, Lyons WS, De Guzman VC et al. Leiomyomata of the esophagus -- An analysis of 838 cases. Cancer 1976;38:2166-2177
4. Federici S, Ceccarelli PL, Bernardi F, Trassinari D, Zanetti G, Tani G, Domini R (1998) Esophageal leiomyomatosis in children: report of a case and review of the literature. Eur J Pediatr Surg 8:358-363
5. Esat M, Banke A, Isin A, et al. Multimodality Diagnostic Imaging of Diffuse Esophageal Leiomyomatosis. Journal of Compute Assist Tomography. 2006;30:100-104
6. Federici S, Ceccarelli PL, Bernardi F, Trassinari D, Zanetti G, Tani G, Domini R (1998) Esophageal leiomyomatosis in children: report of a case and review of the literature. Eur J Pediatr Surg 8:358-363
7. Erone M, Dodero P, Romeo G, Martucciello G, Caffarena PE, Brisigotti M, Toma P, Accone A, Silengo M (1991) Leiomyomatosis of oesophagus, congenital cataracts and hematuria. Report of a case with rectal involvement. Pediatr Radiol 21:578-579
8. G. Azzie, A. Bensoussan, L. Spitz (2003) The association of anorectal leiomyomatosis and diffuse esophageal leiomyomatosis. Pediatr Surg Int 19:424-426
9. Cochat P, Guibaud P, Garcia Torres R, Roussel B, Guarner V, Larbre F (1988) Diffuse leiomyomatosis in Alport syndrome. J Pediatr 113:339-443
10. Cochat P, Guyot C, Antignac C, Pracros JP, Bouvier R, Chappuis JP, Gilbert-Barness E (1993) Pathological case of the month. Alport syndrome and diffuse leiomyomatosis. Am J Dis Child 147:791-792
Keywords: Esophagus, Multiple organ, Benign tumor,

No. of Applicants : 78

▶ Correct Answer : 20/78, 25.6%

- Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
- China Medical University,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
- CHRU lille , France manuel toledano
- Univ. of Miami/Jackson Memorial , United States mantosh rattan
- Hanmaeum Hospital , Korea (South) Kwon Hyoung Kim
- Osaka University , Japan Osamu Honda
- Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
- Changhua Christian Hospital , Taiwan Chia-Fu Tsai
- Asian Heart Institute, Nirman Hitech & Apollo clinic, Mumbai , India Jeshil Shah
- Father Muller Medical College , Mangalore , India nabil mahmood
- mediciti hospitals , India paramjyothi gongati
- Ondokuz Mayis University , Turkey Cetin Celenk
- Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
- kims,narkatpally , India k bhaskar
- Gimpo Woori Hospital , Korea (South) Son Youl Lee
- tantia general hospital , India gurpreet singh
- Homs National Hospital , Syria Rami Abou Zalaf
- Armed Forces Chun-Cheon Hospital , Korea (South) ChaeHun Lim
- All India Institute of Medical Sciences , India Ashish Gupta
- Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal

▶ Correct Answer as Differential Diagnosis : 15/78, 19.2%

- POPOVO HOSPITAL , Bulgaria VLADISLAV RUSINOV
- The Armed Forces HamPyeong Hospital , Korea (South) Bae Geun Oh
- SPS APOLLO HOSPITALS , India LOVKESH MITTAL
- Shiga University of Medical Science , Japan Norihisa Nitta
- Marien Hospital, Hamm , Germany Davis Chiramel
- chp st martin , France Mariotte benoit
- Stroger hospital , United States ky yoo
- E-DA Hospital / I-Shou University , Taiwan Yu-Feng Wei
- IRSA La Rochelle France , France Denis Chabassiere
- Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
- Mallinckrodt Institute of Radiology , United States Naganathan Mani
- Trakya University School of Medicine , Turkey Umut Ugur
- Dongguk University International Hospital , Korea (South) Hee Seok Choi
- All India Institute of medical sciences , India Justin Moses
- Yonsei University college of medicine Severance hospital , Korea (South) Hua Sun Kim

▶ Semi-Correct Answer : 6/78, 7.7%

- McGill University Health Centre , Canada Amr Ajlan
- ROHINI Scans (P) Ltd. , India UMAPATHI MAHESH
- National Taiwan University Hospital , Taiwan Chin-Chung Shu
- clinique de SAVOIE , France, Metropolitan gay-depassier philippe
- Arora Neuro Centre,Ludhiana , India Devendra Kumar
- Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 575 Date 2008-11-03

Discussion

No. of Applicants : 78