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

lNTRODUCTlON

Gastric plexiform fibromyxoma(PF)is a rare mesenchymal tumor,most commonly found in the gastric antrum.Only 121 cases of PF have been reported worldwide,according to a review article published in July 2019[1].This tumor type was first reported in 2007 by Takahashi[2]as plexiform angiomyxoid myofibroblastic tumor because of its pathological feature.Miettinen[3]later redescribed it as‘gastric PF’ in 2009,a name which was also adopted by the World Health Organization’s classification of tumors of the digestive system.The clinical manifestations of gastric PF lack specificity and are mainly presented as slow-onset of nonspecific gastrointestinal discomfort.Few of them have required emergency surgery.Because of its low incidence and nonspecific symptoms,gastric PF is not yet fully understood and often misdiagnosed as gastrointestinal stromal tumor(GIST).Herein,we report a case of gastric PF that ruptured and caused sepsis,with the goal of reminding readers of the infrequent symptoms caused by this rare disease and showing a feasible treatment option.We noted that the patient also had a history of coronavirus disease 2019(COVID-19)vaccinations prior to onset of the disease.In addition,we found more than 13 new cases after reviewing the public literature databases of PubMed and Web of Science[4-15]in Table 1.

CASE PRESENTATlON

Chief complaints

A 26-year-old man was admitted to our hospital with complaint of abdominal pain and fever that had persisted over the past half-month,following receipt of the COVID-19 vaccines.

That became the case in 1947 when Branch Rickey, owner of the Brooklyn Dodgers19, informed his team that he was bringing Jackie Robinson up from the minors-the first black man to play in the majors. Rickey wanted Robinson s talent at the plate and his speed on the base paths. Some players circulated a petition stating they would not take the field with a black man. Pee Wee Reese, the Dodgers shortstop and team captain, refused to sign and tore up the petition, effectively putting an end to a stupid and ignorant idea that was wrong, as well as bad for the team.

History of present illness

On January 11,2021,the patient developed right upper abdominal pain and fever(37.8 °C)5 h after COVID-19 vaccination.During the first 11 d,he did not receive any treatment for the abdominal pain(deemed ‘tolerable’)nor the intermittent low-grade fever.However,his symptoms progressively worsened,to the extent that his tem-perature rose to 39.5 °C and he experienced melena.

The patient first went to the local hospital for medical treatment on January 22,2021.Blood panel and computed tomography(CT)examinations were performed.Signs of infection were detected and a mass was found in the gastric antrum.Because the cause of the infection was unclear,the patient did not receive surgery at the local hospital but did receive oral cephalosporin antibiotics and symptomatic treatment.However,his symptoms were not relieved,so he was referred to our hospital on January 26,2021 for further treatment.After hospitalization,we upgraded the patient’s anti-infective therapy and decided to perform emergency surgery as the care staff noted the patient to have developed a confused state and signs of infectious shock on January 27,2021.

History of past illness

The patient had no significant medical history.

Personal and family history

The patient had no significant family history of any malignancy.

Physical examination

On January 26,2021,the physical examination made upon admission showed tenderness in the right upper abdomen without rebound pain and no palpable lump.Murphy's sign was positive but no signs of jaundice were noted.Basic clinical findings were temperature of 39.6 °C,blood pressure of 153/84 mmHg,heart rate of 126/min,and respiration of 25/min.Findings from other physical examinations were unre-markable.

Laboratory examinations

On January 22,2021,the patient’s blood examination(preformed in the local hospital)indicated an increased white cell count[13.01 × 10/L;reference range:(4.0-10.0)× 10/L]and neutrophil ratio(69.6%;reference range:50%-75%).

Due to the rarity of this disease,the pathology report was provided for consultation by another tertiary hospital;ultimately,the same opinion was given.On gross pathological examination,an 11 cm × 7 cm ×5 cm tumor could be seen on the serosa of gastro-duodenal junction(Figure 3A).The tumor was elastic in texture and its cut surface was gray-red,with some necrotic rupture and cystic degeneration visible.Also,a rough change was seen on the mucosa where the tumor was located.Microscopically,the hematoxylin/eosin-stained pathological sections’ view revealed the tumor to be composed of spindle cells with clarified cytoplasm,accompanied by myxoid stroma and arborizing blood vessels(Figure 3B).The tumor cells were seen to have infiltrated the mucosa,muscularis,and subserosa,but no apparent atypia of tumor cells was observed.No tumor tissue was found in the resected lymph node(0/1)nor at the gastric and duodenal margins.The pathology of the resected gallbladder showed chronic inflammatory changes without tumor infiltration.Immunohistochemically,the spindle cells were positive for smooth muscle actin and succinate dehydrogenase subunit B(SDHB),and weakly-positive staining was observed for desmin(Figure 3C).Immunohistochemistry staining was negative for S-100,DOG-1,CD10,and β-catenin(Figure 3D).CD34 was positive in the capillary network but was negative in tumor cells.CD117 was positive in mast cells but negative in tumor cells(Figure 3E).The Ki-67 proliferative index was ＜ 1%.In summary,the pathological presentation was deemed to be more consistent with a diagnosis of gastric PF.

On January 26,2021(the day of admission to our hospital),the patient’s blood examination indicated a white cell count of 14.61 × 10/L and neutrophil ratio of 84.2%.In addition,we detected remarkable increases in total bilirubin(31.0 mmol/L;reference range:1.7-20 μmol/L),erythrocyte sedimentation rate(58 mm/h;reference range:0-15 mm/h),and hs-C-reactive protein(332.41 mg/L;reference range:＜5 mg/L).Blood culture produced no bacterial growth.Tumor markers,such as carcinoembryonic antigen,alpha-fetoprotein,cancer antigen-199 and cancer antigen-125,were all within normal range.

Imaging examinations

PF is a rare mesenchymal tumor,with limited reports worldwide.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[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].From the literature with clinical manifestations available for 108 cases,86 patients presented as nonspecific gastrointestinal complaints or gastrointestinal hemorrhage,which could indicate that these are the most common symptoms of gastric PF.These manifestations were also reflected in the current case,but the patient’s difficult-to-control infection caused by the ruptured gastric PF was a more notable symptom,which is rare in the disease,causing doctors to face more trouble in the present case.Indeed,it was the infectious shock condition that threatened the patient’s life and forced us to perform emergency surgery.

COVID-19 vaccination has proven to be an effective measure to reduce mortality and contain the spread of COVID-19.In our case,we noted that the patient had a history of COVID-19 vaccination(inactivated vaccine produced by “Beijing Biology”)prior to the acute onset of the disease.We attempted to investigate their connection,if any,but we did not find any evidence of a causal relationship between them.We have also found no reports of abdominal infections among the adverse reactions to this vaccine.We speculate that this may just be a coincidence.We present this pheno-menon here,but whether COVID-19 vaccination was involved in the acute onset of gastric PF deserves further research.

Gastric PF that manifests as tumor rupture combined with pyemia is rare,and timely surgery is critical for optimal prognosis of these patients.

Gastroscopy

On the day of admission,an ulceration(approximately 1.5 cm × 1.0 cm)was detected in the gastric antrum mucosa during the gastroscopy but no obvious mass was reported.The gastric antrum was deformed and stenosis of bulbus duodeni was observed(Figure 2).

FlNAL DlAGNOSlS

On January 23,2021,a second blood examination found the white cell count to have increased to 25.7× 10/L and neutrophil ratio to 82.5%.

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TREATMENT

After routine preoperative preparation,the patient underwent emergency surgery.During the laparoscopic exploration,it was noted that a huge mass(approximately 9 cm × 12 cm × 5 cm)with thick purulence wrapped the distal stomach and the proximal duodenum.The mass was closely adhered to the gallbladder,with unclear demarcation.Necrotic rupture at the mass and abscess formation were observed intraoperatively.Pus was visible in the pelvic and abdominal cavities.Considering the size of the tumor,we eventually adopted laparotomy to resect the mass,and we performed the distal partial gastrectomy,duodenal bulbectomy,and cholecystectomy.The digestive tract was reconstructed in a Billroth II anastomosis.Repeated flushing of the abdominal cavity with physiological saline was conducted,and a drainage tube was placed before abdomen closure.

Frozen-specimen pathological examination of the resected tissues indicated a spindle cell tumor,consistent with the appearance of GIST cells.The patient received anti-infection therapy and advanced care after surgery but no chemotherapy or radiotherapy.

In terms of the auxiliary examination of gastric PF,CT has always been an important examination in most case reports.Of the 13 additional cases,10 of the 11 underwent CT examinations,excluding the 2 cases of PF found by review of previous pathology reported by Arslan[12].It was also CT that enabled us to find the mass in the case we report herein.Gastroscopy allows direct observation of the gastric mucosa.However,we did not see an observable mass in the patient’s gastric mucosa during gastroscopy.We only observed ulceration and a deformed digestive tractgastroscopy,while the presence of a large mass was confirmed by CT.The later pathological examination corroborated the gastroscopic findings.This may suggest that PF could grow out of the intestinal wall and form a huge mass but with rare changes in gastric mucosa.Therefore,we should be alert to the possibility of an extra-intestinal neoplasm,even if only tiny changes in the mucosa are observed during the gastroscopy examination of the gastric PF.

OUTCOME AND FOLLOW-UP

Following an uneventful recovery,the patient was discharged from the hospital on February 10,2021.On the 160day after surgery,he attended follow-up for routine blood test,tumor marker examination,and enhanced CT scan(Figure 4).No signs of tumor recurrence were detected.He was continuing to recover well as of press time.

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DlSCUSSlON

On January 25,2021(at the local hospital),enhanced CT revealed a huge soft tissue mass near the region of the gastric antrum,pyloric canal,and duodenal bulb.The mass was compressing the surrounding tissues and had unclear boundaries.A portion of the mass(approximately 34 mm × 16 mm)showed an inhomogeneous enhancement in the arterial phase(Figure 1).

In known cases,because of the slow-onset process,patients have rarely presented with severe infections or required emergency surgery for gastric PF.We only found a single definitive case[1]of emergency surgery for gastric perforation and a single case[16]showing evident manifestation of infection.As such,this may be the first case report worldwide of emergency surgery performed for ruptured gastric PF with severe infection.The nonspecific and uncommon symptoms of this rare disease pose a challenge in diagnosis and tend to delay treatment.Thus,gastric PF,especially huge gastric PF,may also pose a risk of rupture,necrosis,and serious infection for potentially life-threatening conditions.Timely surgery is the treatment approach that should be considered first.Otherwise,pyemia and abscess,that may prove difficult to control with medication,will follow.

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As of now,no cases have been found where gastric PF was diagnosed before the pathological examination.In the absence of a specific clinical presentation,path-ological examination remains the only means of confirming the disease.However,the rarity of the disease poses a diagnostic challenge,especially to inexperienced pathologists.In our case,although frozen pathology was routinely performed intraoperatively,we still mistook the mass for a GIST until the paraffin-embedded section pathology report.We need to distinguish between gastric PF and GIST,which is the most common mesenchymal tumor of the gastrointestinal tract.In addition to the characteristic view under the microscope,immunohistochemistry is an important method to differentiate gastric PF from GIST.Including the 13 new cases published after the study by Su[1],no detection of DOG-1 or CD117,which are both markers for GIST,were reported for the tumor cells.This case did not overturn the previous immunohistochemical findings of PF.In fact,they still may be the most practical markers to differentiate gastric PF from GIST.Furthermore,it is noteworthy that SDHB was positive in our case,and this test item was the first to be reported in PF cases.Negative SDBH has been identified in SDHdeficient GIST,pheochromocytoma,neuroblastoma[17],and so on.SDHB may also allow PF to be distinguished from some primary tumors,but determination of its significance will require further research.

After hospitalization,as the abdominal pain and confused state intensified,we reassessed the condition of the abdomen with an emergency CT scan on January 27,2021.The imaging findings were similar to previous ones,namely a tissue mass(approximately 91 mm × 77 mm × 115 mm)with heterogeneous density was found in the hepatogastric space.It was poorly demarcated from the gastric antrum,and no free air was detected(Figure 1).

CONCLUSlON

Am I the egg that starts with a malleable6 heart, but changes with the heat? Did I have a fluid spirit but, after a death, a breakup or a financial hardship, does my shell look the same, but on the inside am I bitter and tough with a stiff spirit and a hardened heart? Or am I like the coffee bean? The bean actually changes the hot water, the very circumstance that brings the pain. When the water gets hot, it releases the fragrance7 and flavor. If you are like the bean, when things are at their worst, you get better and change the situation around you. When the hours are the darkest and trials are their greatest, do you elevate to another level? How do you handle adversity? Are you a carrot, an egg or a coffee bean?

ACKNOWLEDGEMENTS

The authors wish to acknowledge the work of staff at The First Affiliated Hospital,Sun Yat-sen University,Guangzhou,China(a tertiary hospital)for their review and confirmation of our patient’s diagnosis.

But at evening she came all at once to the green lawn where the wretched little hut stood on its hens legs. The wall around the hut was made of human bones and on its top were skulls23. There was a gate in the wall, whose hinges were the bones of human feet and whose locks were jaw-bones set with sharp teeth. The sight filled Vasilissa with horror and she stopped as still as a post buried in the ground.

FOOTNOTES

Zhang R designed the report and drafted the manuscript;Huang KB and Chen ND collected patient’s clinical data;Xia LG worked with the pathologists in our hospital to determine the pathological diagnosis after thorough discussion;Xia LG analyzed the data and provided recommendations for the manuscript;and all authors read and approved the final manuscript.

Informed written consent was obtained from the patient for publication of this report and any accompanying images.

The authors declare that they have no conflict of interest.

The authors have read the CARE Checklist(2016),and the manuscript was prepared and revised according to the CARE Checklist(2016).

This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers.It is distributed in accordance with the Creative Commons Attribution NonCommercial(CC BYNC 4.0)license,which permits others to distribute,remix,adapt,build upon this work non-commercially,and license their derivative works on different terms,provided the original work is properly cited and the use is noncommercial.See:https://creativecommons.org/Licenses/by-nc/4.0/

China

The backdoor opened. My husband walked outside. I love you, he said. I was wondering where you were. I chuckled11, as he came over and kissed me, then turned around and went back inside. Where s the quiet time? I asked God. My heart fluttered. There was no pain, only a beat that interrupted me yet again. This is impossible, I thought. There s no time to be still and to know that God is with me. There s too much going on in the world and entirely12 too much activity all around me.

Rui Zhang 0000-0001-6221-3464;Li-Gang Xia 0000-0002-6481-8405;Kai-Bin Huang 0000-0002-7956-3564;Nan-Di Chen 0000-0003-0849-014X.

Wang JJ

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Wang JJ