Solitary infantile gastrointestinal myofibroma: case report (original) (raw)
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Infantile myofibromas obstructing opposite ends of the gastrointestinal tract
Journal of Pediatric Surgery, 2013
Myofibromas are benign congenital tumors of soft tissue that can present at birth or during infancy in solitary or multicentric forms. Visceral myofibromas are rarely reported, but are typically symptomatic due to involvement of vital structures. We present two cases of congenital myofibromas, one obstructing the proximal esophagus and the other obstructing the distal rectum. Lessons learned from the treatment of these two patients are shared and the pertinent literature is reviewed.
Pathology International, 2001
A 2-year-old girl was admitted to the Department of Pediatric Gastroenterology and Nutrition, Ege University, for intermittent vomiting and diarrhea for the previous 4 months. Routine investigation demonstrated a moderate malnutrition according to the Gomez classification, and barium enema examination showed small intestinal obstruction . During laparotomy, an annular mass surrounding the jejunum was discovered and a jejunal resection was performed.
Intra-abdominal infantile inflammatory myofibroblastic tumors: A report of three cases
Journal of Indian Association of Pediatric Surgeons, 2014
Inflammatory myofibroblastic tumor occurring at intra-abdominal sites in children can be confused with malignancy because of its large size and location. It is a tumor classified as 'intermediate' between benign and malignant, but usually benign, with a strong tendency for recurrence. Treatment is surgical excision. Here, we present a brief outline of three such cases presenting as abdominal mass in infants.
Abdominal inflammatory myofibroblastic tumours in children
British Journal of Surgery, 1992
Inflammatory myofibroblastic tumours ( inflammatory pseudotumours) occurring at intra-abdominal sites in children have rarely been described. This paper reports three patients with this tumour, two of whom presented with fever, anaemia and an abdominal mass, the third with chronic duodenal obstruction. All had experienced significant weight loss. At operation, each had a large fibrous tumour (7–18 cm in diameter) originating from the transverse mesocolon, small bowel mesentery and duodenum respectively. Intraoperative frozen section histological examination in one patient was misinterpreted as a sarcoma. All the lesions were judged to have been completely excised, but one was ruptured during operation and the patient subsequently developed recurrent tumour nodules. Abdominal inflammatory myofibroblastic tumours are rare. They may be suspected before operation but their clinical, radiological and pathological features may be confused with those of malignancy. Complete excision is necessary to avoid local recurrence.
Inflammatory Myofibroblastic Tumor: Encounter with A Rare Tumour in Pediatric Age Group
Crimson Publishers, 2019
Inflammatory myofibroblastic tumor, a rare benign tumor, occurs at any site in the body, is more common in pediatric age group than adult. Among them, inflammatory myofibroblastic tumors arising from stomach and mesentery are rare entity. We report two cases A. 9 years old boy who suffered from dysphagia, vomiting and weight loss. Barium swallow examination revealed narrowed distal esophagus up to lower gastro-esophageal junction with bird-beak appearance. UGI Endoscopy revealed narrowed lower esophagus. During operation, the mass was found to be in fundus of stomach obstructing gastro-esophageal junction and total radical gastrectomy was done and histopathology report traced inflammatory myofibroblastic tumor. B. 7 years old boy presented with recurrent (two times operated) ill-defined, periumbilical abdominal mass. Previous tissue biopsy was non-conclusive. Abdominal Contrast CT Scan revealed heterogenous mass in epigastric and umbilical region encasing Superior Mesenteric Artery (SMA), which could not be separated from small bowel loops. During operation, the tumor was found to be arising from root of mesentery, encasing SMA & Head of Pancreas and adhere with almost middle ½ of small intestine. Debulking of mass along with resection anastomosis of small intestine was realized and histopathology report traced inflammatory myofibroblastic tumour.
Histopathology, 2012
Solitary, multifocal and generalized myofibromas: clinicopathological and immunohistochemical features of 114 cases Aims: To report a large series of solitary and multiple myofibromas with systematic clinicopathological correlations. Methods and results: We report on 114 patients with myofibromas, 97 of which were solitary and 17 multifocal. The age at presentation ranged from newborn to 70 years. All multifocal myofibromas and 91% of solitary myofibromas occurred in children. The head and neck region was the most common site (n = 43), followed by the trunk (n = 24), lower limbs (n = 14), upper limbs (n = 11), and viscera (n = 4). Solitary and multifocal myofibromas stained positively for smooth muscle actin (SMA) in 95% and 92% of cases, muscle-specific actin (MSA) in 75% and 50% of cases, and desmin in 10% and 14% of cases, respectively. Regressive features were seen in 34 solitary myofibromas and in nine multifocal myofibromas. Most patients were treated with complete excision (n = 79) or partial excision (n = 12). There were no recurrences after treatment. Conclusions: Solitary and multiple myofibromas are benign tumours that predominantly occur in infancy and childhood. Myofibromas occur especially in the head and neck region, and are characterized by SMA and, to a lesser extent, MSA expression. The clinical course is self-limiting, and local excision appears to be sufficient.
Journal of Paediatric Surgeons of Bangladesh, 2015
Inflammatory myofibroblastic tumour (IMT) occurring at intraabdminal sites in children has rarely been described. Inflammatory pseudotumour is a soft tissue lesion that may be confused with a sarcoma. It is abbreviated as IMT. Inflammatory myofibroblastic tumour, also known as soft tissue tumours ,atypical fibromyxoid tumours, pseudosarcomatous fibromyxoid tumour ,plasma cell granuloma, pseudosarcomatous myofibrotic proliferation, post-operative spindle cell nodules. In this paper, we describe a case of inflammatory myofibroblastic tumour (IMT) with an unusual constellation of clinical, pathological findings. A 10-year-old girl had an 7-cm intraabdominal mass accompanied by severe anemia, fever, constipation, weight loss, thrombocytosis, elevated erythrocyte sedimentation rate. Laparotomy was performed. The final pathologic diagnosis was IMT. At the most recent follow up (12months) after excision of the tumour, the patient was symptom-free and there was no evidence of tumour recurrence.
Abdominal Inflammatory Myofibroblastic Tumor: Report on
2011
The Abdominal Inflammatory Myofibroblastic Tumor (AIMT) is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT.
Congenital infantile myofibroma; case report and review of literature
Human Pathology: Case Reports, 2020
Infantile myofibroma or Infantile myofibromatosis is rare benign tumour though it is the most common mesenchymal tumour occurring in neonates and infancy. Most of the patients present during first year while upto 60% of infantile myofibromas are congenital. Myofibromatosis was first described by Stout and was known as congenital generalized fibromatosis in 1954 [1]. The name Infantile myofibroma was coined by Chung and Enzinger in 1981 and they classified infantile myofibroma as distinct lesion from fibromatosis [2]. Smith et al. and Daimaru et al. in 1989 coined the terms "myofibromas" and "myofibromatosis for solitary and multicentric tumours, respectively [3,4]. The disease is known to be associated with mutations in PDGFRB and NOTCH3 gene [5,6]. Multiple heterozygous mutations in PDGFRB gene on chromosome 5q32 and heterozygous mutation in the NOTCH3 gene alterations are known [7]. The most common mutation in PDGFRB was found to be p.R561C (C.1681C > T) while that in NOTCH3 was L1519P (C.4556 T > C). PDGFRB mutations are more common with multicentric tumours as compared to solitary tumours [8]. NOTCH3 mutations are seen in 11% of children with infantile myofibromatosis. These tumours may present sporadically or may have autosomal dominant inheritance with variable expressivity. This article describes a case of a 05 months old patient who presented with a scalp tumour. The aim is to increase awareness about this rare entity. Written informed consent for publication was obtained from the father.