Dataset Information

Inflammatory myofibroblastic tumors of the pancreas in children: A case report and literature review.

ABSTRACT: Inflammatory myofibroblastic tumors are an uncommon neoplasm, which are very rarely located in the pancreas. Clinically and radiologically, this rare pancreatic tumor presents as an abdominal mass lesion that mimics other pancreatic tumors, and should therefore be considered in the differential diagnosis of pancreatic tumors.The 15-year-old boy complained of abdominal pain over the left upper quadrant with intermittent fever for 7 days. Abdominal sonography revealed one cystic lesion with a hyperechoic component in the left upper quadrant of the abdomen. Surgical excision was performed and postoperative findings indicated a pancreatic tail tumor. The pathology indicated inflammatory myofibroblastic tumors. To our knowledge, this patient is a unique case as the tumor was located in the pancreatic tail only, sparing the body.The patient underwent tumor resection and segmental resection of the transverse colon with simple closure. The patient had no evidence of disease recurrence at 3 years follow-up.Inflammatory myofibroblastic tumors of the pancreas in children are extremely rare. Surgical excision is the standard treatment, and corticosteroids use in children need more large-scale studies.

SUBMITTER: Liu HK

PROVIDER: S-EPMC5266186 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Inflammatory myofibroblastic tumors of the pancreas in children: A case report and literature review.

Liu Hsien-Kuan HK Lin Yung-Cheng YC Yeh Ming-Lun ML Chen Yaw-Sen YS Su Yu-Tsun YT Tsai Ching-Chung CC

Medicine 20170101 2

<h4>Introduction</h4>Inflammatory myofibroblastic tumors are an uncommon neoplasm, which are very rarely located in the pancreas. Clinically and radiologically, this rare pancreatic tumor presents as an abdominal mass lesion that mimics other pancreatic tumors, and should therefore be considered in the differential diagnosis of pancreatic tumors.<h4>Clinical findings and diagnosis</h4>The 15-year-old boy complained of abdominal pain over the left upper quadrant with intermittent fever for 7 days ...[more]

PMID: 28079824

Similar Datasets

Project description:BACKGROUND:Inflammatory myofibroblastic tumors (IMTs) of the thyroid are extremely rare soft-tissue tumors. In the literature, IMTs are sometimes called plasma cell granulomas (PCGs) or inflammatory pseudotumors, which often causes ambiguity. To date, 17 cases of PCGs and five cases of thyroid IMTs have been reported. These cases reveal that IMTs of the thyroid are often negative for the anaplastic lymphoma kinase (ALK-1) gene. To provide further information on this rare lesion, we present a case of an ALK-1-positive thyroid IMT and a review of IMTs of the thyroid. CASE PRESENTATION:A 34-year-old Chinese woman presented with a painless neck mass that had persisted for over a month. Ultrasonography revealed a 4.28 × 2.53 cm2 hypoechoic mass, in the left lobe of the thyroid gland. Serum levels of thyroglobulin and anti-thyroglobulin antibodies were high. Subsequently, left lobectomy was performed. Macroscopically, the lesion was a gray-brown nodular mass with a partial envelope. Histologically, two different lesion types were observed. The first lesion showed classic spindle cell proliferation, with spindle cells arranged in fascicles, accompanied by mature inflammatory cells. The other lesion showed a large number of infiltrating lymphocytes, with lymphoid follicles in the remaining thyroid gland, which was atrophic. Immunohistochemical staining showed that the spindle cells were negative for CK19, CyclinD1, Gelectin-3, EMA, CD34, S100, Bcl-2, and STAT-6, but strongly positive for ALK-1, vimentin, and TTF1. CK was focally expressed, and the Ki-67 index was 5%. A diagnosis of IMT was proposed according to immunohistochemical findings and morphology. Hashimoto's thyroiditis was confirmed according to serum levels of thyroglobulin and anti-thyroglobulin antibodies and morphology. The patient did not receive adjuvant therapy. She remained alive without disease recurrence for 10 months after lobectomy. CONCLUSIONS:IMTs should be considered in the diagnosis when spindle cell proliferation accompanied by mature inflammatory cells is observed, spindle cells are mildly atypical, and myofibroblast differentiation is present in the thyroid. A uniform diagnostic term is crucial to avoid ambiguity. Clinicians and pathologists should be aware of the necessity for long-term follow-up, especially in ALK-positive cases. The therapeutic potential of ALK-1 positivity should be explored further.

Dataset Information

Inflammatory myofibroblastic tumors of the pancreas in children: A case report and literature review.

Publications

Inflammatory myofibroblastic tumors of the pancreas in children: A case report and literature review.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets