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Gastric plexiform fibromyxoma arising in the cardia in an adolescent male. A rare tumor with unusual presentation

Awrad Nasralla, MD, Mufeed Alwabari, MD, Osama Alsaif, MD, Samir S Amr, MD. King Fahad Specialist Hospital -Dammam

Introduction: The objective is to report the sixth case of gastric plexiform fibromyxoma in the pediatric population with unusual location in the cardia. It is an exceedingly rare mesenchymal tumor, that usually affects adults. It has non-specific clinical manifestations. Treatment is surgical resection of the tumor; however, the type of surgery depends on the size, and location of the tumor. Preoperatively, this tumor is usually diagnosed as gastrointestinal stromal tumor (GIST), and the correct diagnosis is made on histopathological examination following surgical resection.

Case presentation: A 16-year-old boy presented with a history of two episodes of hematemesis, without associated other gastrointestinal symptoms. Past medical, surgical, and family histories were unremarkable.

On physical examination, the patient was overweight, looking pale. His vital signs were within normal limits. His abdomen was soft, not tender, nor distended, with no palpable masses. Laboratory investigations revealed low hemoglobin value (8.2 g/ dL). CT scan revealed lobulated submucosal gastric mass at the gastric cardia measuring 4.3 x 4 x 4.7cm.

Upper gastrointestinal endoscopy showed normal esophagus, submucosal mass (4 cm), with deep ulcer at the cardia, the first and second parts of the duodenum were normal (Figure 1).  In addition, EUS was done which demonstrated a submucosal mass at the cardia measuring 5 X 3 cm. It was oval in shape, heterogenous, echogenic, soft, with no appreciable adjacent lymph nodes.

The patient underwent laparotomy, with wedge resection of the mass and primary anastomosis. Postoperative course was uneventful. Pathological examination of the specimen revealed gastric plexiform fibromyxoma with the size of 4 X 4.9 X 4.5 cm, margins were free from the tumor.

Discussion: Plexiform fibromyxoma is most commonly found in the gastric antrum (88 %). Other reported locations were duodenal bulb (22%), gastric body (2.6 %), and gastric fundus (1.3 %). There is one report of plexiform fibromyxoma located in esophagus. Our case is the first one to report the tumor at the gastric cardia near the gastroesophageal junction in a child. In this case, gastric plexiform fibromyxoma was not expected due the age of the patient and the location of the tumor.

Conclusion: Plexiform fibromyxoma can be found in children and it can be located anywhere in the stomach. Images and endoscopy aid in assessing the location, and the size of the tumor, which helps to decide the best surgical technique. However, the diagnosis usually established after histopathogical examination of the tumor following surgery.

Figure 1: Endoscopic picture 


Presented at the SAGES 2017 Annual Meeting in Houston, TX.

Abstract ID: 93910

Program Number: P493

Presentation Session: Poster Session (Non CME)

Presentation Type: Poster

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Post Views: 12

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