Plexiform angiomyxoid myofibroblastic tumour of the duodenum: a rare entity

Banerjee, Niladri; Gupta, Swapnil; Dash, Suvashis; Ghosh, Saswata

doi:10.1136/bcr-2015-210004

Cited by 15 publications

(16 citation statements)

References 13 publications

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“…It typically arises in the gastric antrum; exceptional extragastric cases have been described in the esophagus, duodenum, jejunum, gallbladder and, possibly, in the colon. [ 22 , 26 , 27 , 30 , 33 ] PAMT characteristically features a plexiform architecture, with myxoid nodules located in the gastric muscularis propria, often ulcerating the overlying mucosa, composed of ovoid cells with indistinct cytoplasm; a prominent capillary network is invariably present. At IHC, PAMT is positive for α-smooth muscle actin (α-SMA) and, sometimes, for desmin and/or caldesmon, consistently with myofibroblastic differentiation.…”

Section: Introductionmentioning

confidence: 99%

Unusual focal keratin expression in plexiform angiomyxoid myofibroblastic tumor

et al. 2016

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Background:Plexiform angiomyxoid myofibroblastic tumor (PAMT), also known as plexiform fibromyxoma, is a rare distinctive benign intramural tumor, typical of gastric antrum, commonly causing mucosal ulceration with upper gastrointestinal bleeding and anemia, effectively treated by complete surgical resection usually accomplished by distal gastrectomy.Methods and Results:We herein report a 47-year-old man presenting with a syncopal episode, regurgitation and epigastric discomfort, bearing a gastric antral myxoid plexiform tumor positive for α-smooth muscle actin, vimentin and, partially, for caldesmon, desmin, and CD10; CD117, DOG1, CD34, S100, CAM5.2, CK20, CK7, EMA, p53, CDX2, chromogranin A, synaptophysin, anaplastic lymphoma kinase, Melan-A, and HMB-45 were all negative. All these features are typical of PAMT. Of note, focal positivity for AE1/AE3 and pan-CK KL1 was also present.Conclusions:The finding of a focal keratin expression in PAMT contributes to enlarge the immunophenotypic spectrum of this tumor type and is relevant for avoiding presurgical misdiagnoses which could ultimately lead to inappropriate overtreatment of patients with PAMT.

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Section: Introductionmentioning

confidence: 99%

Unusual focal keratin expression in plexiform angiomyxoid myofibroblastic tumor

et al. 2016

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“…In most areas, the tumor cells are arranged loosely. Immunohistochemical staining shows that plexiform fibromyxoma cells are positive for SMA[17], focally positive for CD10, and negative for cytokeratin, CD117, DOG-1, desmin, S-100, epithelial membrane antigen, and CD34[1,2,6,7]. Mitoses are rare (up to 7/50 HPF).…”

Section: Discussionmentioning

confidence: 99%

“…The most common presenting symptoms are anemia, hematemesis, and hematochezia. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum[6]. We here report a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal.…”

Section: Introductionmentioning

confidence: 89%

Plexiform fibromyxoma of the small bowel: A case report

Zhang

Xiang

et al. 2018

WJCC

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BACKGROUNDPlexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum.CASE SUMMARYWe report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1 (discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination.CONCLUSIONPlexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.

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“…The tumor shows a similar incidence in both genders, and it occurs over a wide age range (7–75 years) [ 4 6 ]. The majority of PAMTs were found in the stomach, with only one case reported in the duodenum [ 7 ]. Most gastric PAMTs occurred in the gastric antrum, but they could be located anywhere in the stomach [ 4 6 ].…”

Section: Discussionmentioning

confidence: 99%

Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: a Rare Case

Kim

Choi

et al. 2017

J Gastric Cancer

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Plexiform angiomyxoid myofibroblastic tumor (PAMT) of the stomach is a very rare mesenchymal tumor of the gastrointestinal tract. We report a case of asymptomatic gastric PAMT that was pathologically confirmed after surgical resection. The tumor had a multinodular plexiform growth pattern, bland-looking spindle cells, and an Alcian blue-positive myxoid stromal matrix rich in small blood vessels. Immunohistochemistry analysis revealed that the tumor cells of the PAMT were positive for smooth muscle actin (SMA) and negative for c-kit, CD34, S-100 protein, epithelial membrane antigen (EMA), and desmin. PAMT should be differentiated from other submucosal tumors of the stomach by immunohistochemical findings. Considering the benign features of this tumor, observation without resection may be an option for the treatment of PAMT if the tumor is asymptomatic.

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Plexiform angiomyxoid myofibroblastic tumour of the duodenum: a rare entity

Cited by 15 publications

References 13 publications

Unusual focal keratin expression in plexiform angiomyxoid myofibroblastic tumor

Unusual focal keratin expression in plexiform angiomyxoid myofibroblastic tumor

Plexiform fibromyxoma of the small bowel: A case report

Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: a Rare Case

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