Gastric Plexiform Fibromyxoma Arising in the Cardia in an Adolescent Male: A Rare Tumor with an Unusual Location

Nasralla, Awrad; Alwabari, Mufeed; Al-Saif, Osama; Amr, Samir S.

doi:10.1155/2020/9037960

Cited by 5 publications

(6 citation statements)

References 17 publications

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“…PFs have been reported as either case reports or small case series. To date, a total of 130 PF cases have been reported in the English literature[ 1 , 6 , 16 - 22 ]. Given the fact that only nineteen cases had been described by 2010 (approximately three years after the first description of the entity), this remarkable increase in its incidence in a short period of time may be attributable to the increased awareness of PF by clinicians and pathologists[ 1 , 7 ].…”

Section: History and Nomenclaturementioning

confidence: 99%

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Plexiform fibromyxoma: Review of rare mesenchymal gastric neoplasm and its differential diagnosis

Arslan

et al. 2021

WJGO

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Plexiform fibromyxoma (PF) is a very rare mesenchymal neoplasm of the stomach that was first described in 2007 and was officially recognized as a subtype of gastric mesenchymal neoplasm by World Health Organization (WHO) in 2010. Histologically, PF is characterized by a plexiform growth of bland spindle to ovoid cells embedded in a myxoid stroma that is rich in small vessels. The lesion is usually paucicellular. While mucosal and vascular invasion have been documented, no metastasis or malignant transformation has been reported. Its pathogenesis is largely unknown and defining molecular alterations are not currently available. There are other mesenchymal tumors arising in the gastrointestinal tract that need to be differentiated from PF given their differing biologic behaviors and malignant potential. Histologic mimics with spindle cells include gastrointestinal stromal tumor, smooth muscle tumor, and nerve sheath tumor. Histologic mimics with myxoid stroma include myxoma and aggressive angiomyxoma. Molecular alterations that have been described in a subset of PF may be seen in gastroblastoma and malignant epithelioid tumor with glioma-associated oncogene homologue 1 ( GLI1 ) rearrangement. The recent increase in publications on PF reflects growing recognition of this entity with expansion of clinical and pathologic findings in these cases. Herein we provide a review of PF in comparison to other mesenchymal tumors with histologic and molecular resemblance to raise the awareness of this enigmatic neoplasm. Also, we highlight the challenges pathologists face when the sample is small, or such rare entity is encountered intraoperatively.

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Section: History and Nomenclaturementioning

confidence: 99%

“…Data regarding the radiologic features of PF are limited in the literature due to its rarity[ 28 ]. Imaging findings may be suggestive of another mesenchymal tumor such as GIST[ 22 ]. A small tumor can be missed on computed tomography (CT) and may require invasive imaging modality such as capsule endoscopic examination[ 4 ].…”

Section: Imagingmentioning

confidence: 99%

Plexiform fibromyxoma: Review of rare mesenchymal gastric neoplasm and its differential diagnosis

Arslan

et al. 2021

WJGO

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“…We conducted a database search of PubMed and Web of Science using the terms of “plexiform fibromyxoma” and “plexiform angiomyxoid myofibroblastic tumor”, as of June 2021. at least 13 additional cases were found, published after the review study by Su et al [ 1 ], raising the number of reported cases to 134 worldwide. Of the 13 new cases, 9 patients presented with abdominal signs or bleeding symptoms[ 4 , 6 , 7 , 9 - 12 , 15 ] in Table 1 . Others were detected on medical examination performed for other reasons, such as hematuria[ 12 ], pharyngeal discomfort[ 13 ], and weight loss[ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Huge gastric plexiform fibromyxoma presenting as pyemia by rupture of tumor: A case report

Zhang¹,

Xia²,

Huang³

et al. 2022

WJCC

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BACKGROUND Plexiform fibromyxoma (PF) is a rare mesenchymal tumor, with limited case reports worldwide. Common clinical symptoms are abdominal discomfort and bleeding signs, which frequently present slow-onset in reported cases. Herein, we report a case of gastric PF presenting as acute onset and with pyemia accom-panying tumor rupture. We resected the tumor as well as the distal gastric, bulbus duodeni and gallbladder for treatment in emergency surgery. Notably, before the onset of the disease, the patient received coronavirus disease 2019 (COVID-19) vaccines. CASE SUMMARY A 26-year-old man was admitted to our hospital, due to abdominal pain and fever after having received COVID-19 vaccines. Laboratory examination indicated severe sepsis. Computed tomography scan revealed a large mass in the abdomen. Deformation of the gastrointestinal tract was seen during gastroscopy. After failure of anti-infective treatment and symptoms of shock developed, he received an emergency surgery. We found a huge and partly ruptured mass, with thick purulence. Microscopically, the mass was composed of spindle cells with clarified cytoplasm, accompanied by myxoid stroma and arborizing blood vessels. Immunohistochemistry showed the tumor cells as positive for smooth muscle actin and succinate dehydrogenase subunit B but negative for DOG-1 and CD117. Finally, the patient was diagnosed with gastric PF and discharged from the hospital. CONCLUSION Gastric PF manifesting as tumor rupture combined with pyemia is rare. Timely surgery is critical for optimal prognosis.

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“…The estimated number of pediatric cases ( ≤ 18 years old) of plexiform fibromyxoma is "12" to date, taking into account the published cases in English literature. [7][8][9][10] In this study, we present the case of a 3-month-old infant with a definitive diagnosis of plexiform fibromyxoma in the jejunum which presented as an intestinal subocclusion.…”

mentioning

confidence: 99%

“…This tumor is exceptionally rare in pediatric patients. The estimated number of pediatric cases (≤18 years old) of plexiform fibromyxoma is “12” to date, taking into account the published cases in English literature 7–10 …”

mentioning

confidence: 99%

Pediatric Plexiform Fibromyxoma: A Case Report

Bugeda Gómez,

Costa-Roig,

Montecino Romanini

et al. 2024

Journal of Pediatric Hematology/Oncology

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The plexiform fibromyxoma is a rare mesenchymal tumor in adults that generally originates in the antrum of stomach, being its occurrence in pediatric patients exceptional. It was classified as a distinct entity by World Health Organization in 2010. No recurrences and metastases have been documented in many of the reported patients to date, being the surgical treatment curative. We report the case of a 3-month-old infant who presented to the emergency department with an episode of intestinal subocclusion requiring an emergent surgery. During the surgical intervention, a mass was identified in the jejunum, causing partial occlusion of its lumen. The surgical pathology report revealed an infiltrative tumor composed of spindle-shaped cells disposed in a stroma with a plexiform pattern alternating myxoid areas. These findings and the immunohistochemical characteristics of the neoplastic cells led to classify the tumor as a plexiform fibromyxoma. A description of the immunophenotype of this tumor is made and differential diagnosis with other gastrointestinal tumors is also discussed.

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Gastric Plexiform Fibromyxoma Arising in the Cardia in an Adolescent Male: A Rare Tumor with an Unusual Location

Cited by 5 publications

References 17 publications

Plexiform fibromyxoma: Review of rare mesenchymal gastric neoplasm and its differential diagnosis

Plexiform fibromyxoma: Review of rare mesenchymal gastric neoplasm and its differential diagnosis

Huge gastric plexiform fibromyxoma presenting as pyemia by rupture of tumor: A case report

Pediatric Plexiform Fibromyxoma: A Case Report

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