Gastric Fibromatosis: A case report and review of literature from a Tertiary Cancer Center

Amr Abouzid; Mahmoud M Saleh; Gehad A Saleh; Amany Hassan; Omar Hamdy

doi:10.4103/cmrp.cmrp_1_21

CASE REPORT

Year : 2021 | Volume : 11 | Issue : 6 | Page : 284-287

Gastric Fibromatosis: A case report and review of literature from a Tertiary Cancer Center

Amr Abouzid¹, Mahmoud M Saleh¹, Gehad A Saleh², Amany Hassan³, Omar Hamdy¹
¹ Department of Surgical Oncology, Oncology Center, Mansoura University, Egypt
² Department of Radiology, Faculty of Medicine, Mansoura University, Egypt
³ Department of Pathology, Faculty of Medicine, Mansoura University, Egypt

Date of Submission	01-Jan-2021
Date of Decision	14-Oct-2021
Date of Acceptance	08-Nov-2021
Date of Web Publication	31-Dec-2021

Correspondence Address:
Dr. Amr Abouzid
Department of Surgical Oncology, Oncology Center, Mansoura University, 101 Gomhouria St., Mansoura
Egypt

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/cmrp.cmrp_1_21

Abstract

Gastrointestinal fibromatosis is a rare mesenchymal tumor. It has local invasive behaviour with low-metastatic potential. We present a 36-year-old female patient presented to Oncology Center, Mansoura University, Egypt, with accidentally discovered gastric mass by abdominal computed tomography (CT). The mass was adherent to the posterior wall of the gastric fundus, the tail of pancreas and encasing the splenic artery. The patient underwent partial gastrectomy, distal pancreatectomy and splenectomy. Pathological examination of the specimen showed bland spindle cell proliferation, with oval nuclei, scanty eosinophilic cytoplasm. There was no detected mitosis or necrosis or haemorrhage. Immunohistochemistry of tumor cells was positive with β-catenin and SMA, while Desmin, CD117, DOG-1 and S-100 were negative. The patient did not receive any adjuvant therapy. The patient underwent strict periodic follow-up every 3 months with abdominal CT for 6 months after surgery.

Keywords: Gastrectomy, Gastric fibromatosis, Gastric Mesenchymal Tumors

How to cite this article:
Abouzid A, Saleh MM, Saleh GA, Hassan A, Hamdy O. Gastric Fibromatosis: A case report and review of literature from a Tertiary Cancer Center. Curr Med Res Pract 2021;11:284-7

How to cite this URL:
Abouzid A, Saleh MM, Saleh GA, Hassan A, Hamdy O. Gastric Fibromatosis: A case report and review of literature from a Tertiary Cancer Center. Curr Med Res Pract [serial online] 2021 [cited 2022 Aug 12];11:284-7. Available from: http://www.cmrpjournal.org/text.asp?2021/11/6/284/334574

Introduction

Gastrointestinal fibromatosis is a rare tumor that represents about 0.03% to 0.1% of all neoplasms. They are more frequent in the third and fourth decades, especially infertile women. This tumor has a high degree of local invasion and recurrence with low-metastatic potential.^[1] Gastrointestinal stromal tumor (GIST) is the most frequent mesenchymal tumor, but it is still a rare gastrointestinal tumor.^[2] Distinguishing between these two tumors is difficult since they are often confusing clinically, radiologically and even pathologically. Immunohistochemical staining is important to diagnose and differentiate different types of mesenchymal tumors.^[3] The gastrointestinal fibromatosis is usually asymptomatic, and there are no clear guidelines for its treatment.^[4] We present a case of gastric fibromatosis presented with accidentally discovered gastric mass and was considered as a gastrointestinal stromal tumor.

Case Report

A 36-year-old female patient was referred to Oncology Center, Mansoura University on 18 April 2020, with accidentally discovered abdominal mass by abdominal computed tomography (CT) [Figure 1] that demonstrated a well-defined soft tissue mass seen in the lesser sac measuring 37 mm × 49 mm × 43 mm (anteroposterior × transverse × height), respectively, suggestive of GIST. The mass is seen inseparable from the posterior gastric wall, pancreatic tail and invading the splenic artery more than 270° of its circumference. No pathological regional lymph nodes was noticed. Physical examination was free. The patient had a history of hypothyroidism and recent appendectomy 1 month before her admission to our department. The patient had an ultrasound-guided biopsy from the mass that revealed an acellular smear. Laparotomy was done and with the opening of the lesser sac, the mass was adherent to the posterior wall of the gastric fundus, inseparable from the tail and part of the body of the pancreas and encasing the splenic artery. The patient underwent partial gastrectomy, distal pancreatectomy and splenectomy through an open linear stapler and the mass was excised en bloc [Figure 2]. The patient started oral in post-operative day [POD 5] and had mild bilateral pleural effusion which was treated conservatively. The patient was discharged in POD 7. The histological macroscopic analysis reported an irregular mass sized 8 cm × 5 cm, greyish in colour and firm consistency. In the microscopic analysis [Figure 3], there was spindle cellular proliferation formed of bland spindle cells, with oval nuclei, scanty eosinophilic cytoplasm. There was no detected mitosis or necrosis or haemorrhage. Immunohistochemistry of tumor cells was done with positive β-catenin reaction in the cytoplasm of neoplastic cells, and Smooth muscle actin (SMA) had focal positive reaction in neoplastic cells, while Desmin, CD117, DOG-1 and S-100 were negative, suggestive of fibromatosis infiltrating stomach wall, pancreas and spleen. The patient did not receive any adjuvant therapy as tamoxifen as she is nulliparous and seeking fertility and radiotherapy was not indicated for her due to the tumor location in the upper abdomen with vital nearby structures. The patient underwent a post-operative colonoscopy to exclude any colonic polyps as in familial adenomatous polyposis (FAP) or Gardner's syndrome patients. She underwent periodic follow-up every 3 months in which abdominal CT was done with no evidence of tumor recurrence during the whole 6 months after the surgery.

Figure 1: Post-contrast computed tomography abdomen axial, sagittal and coronal reformatted images: Heterogeneously enhanced exophytic posterior gastric wall soft tissue mass (arrowheads) is seen inseparable from the pancreatic tail and distal body. The mass is seen invading the splenic artery (arrows) more than 270° of its circumference. No pathological regional LNs

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Figure 2: Post-operative specimen. Macroscopic view: (a) Anterior view. (b) Posterior view

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Figure 3: (a and b) Interlacing bundles of fibroblasts separated by collagen arranged in fascicular pattern infiltrating the pancreatic tissue (a) and into the gastric muscular wall (b) (H and E, ×40). (c and d) Proliferation of wavy spindled cell having indistinct cytoplasmic borders and bland nuclear features arranged in fascicular to vaguely whorled pattern (H and E, ×200 and × 100) (e and f) Focal positive nuclear reaction for β-catenin in tumor cells (β-catenin, ×400)

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Discussion

Fibromatoses are rare tumors derived from the musculoaponeurotic structures.^[5] It belongs to intermediate locally invasive tumors according to the WHO Classification of Soft Tissue Tumors in 2013.^[6]

The peak age of onset is between 10 and 40 years old, with no obvious gender difference; our patient was 36 years' female. Fibromatosis has three locations: abdominal wall fibromatosis, intra-abdominal and extra-abdominal fibromatosis in the neck, shoulders, limbs, intracranial, intrathoracic, breast, thyroid and liver.^[7] Intra-abdominal fibromatosis has a worse prognosis owing to its proximity to vital organs, with possible grave invasion, aneurysm formation and bowel obstruction.^[8]

Most cases of gastrointestinal fibromatosis occur sporadically; however, 5% are associated with genetic disorders, as in FAP patients or Gardner's syndrome.^[9] Other risk factors that may be associated with this tumor, include pregnancy, trauma, use of oral contraceptives, family history of desmoid tumor and previous abdominal surgery. The pathological features of fibromatosis are spindle-shaped cells. Immunohistochemically, the tumor cells are positive for vimentin and β-catenin, whereas S-100, CD117 and CD34 are negative.^[10]

Gastrointestinal desmoid tumors and fibromatoses are asymptomatic in 30% of the patients as in our patient that was diagnosed accidentally by abdominal CT; however, they may present with intestinal obstruction and hydronephrosis in advanced cases involving the small bowel or the ureters.^[11]

Gastric fibromatosis mimics gastric mesenchymal tumors such as GIST, inflammatory fibroid polyp, schwannoma, leiomyoma, inflammatory myofibroblastic tumor, myofibroma/myofibromatosis, solitary fibrous tumor of the stomach and scirrhous carcinoma of the stomach.

GIST is the most common mesenchymal tumor of the gastrointestinal tract, usually occurs in the old age (ranged 60–65 years). Gastric GIST grossly presents with variable-sized masses. The histology of gastric GIST is diverse, commonly spindle cell type, a few are epithelioid cell type and mixed type. It is positive for CD117 and DOG1, and sometimes for CD34, SMA and S-100 protein. KIT or PDGFRA gene mutations are also be detected.^[12]

An inflammatory fibroid polyp usually occurs in old patients >60 years old. It presents with a polypoidal swelling of about 1.5 cm in diameter. Microscopically, it is composed of loose connective tissue, spindle-shaped fibroblasts and inflammatory cells such as lymphocytes and eosinophilic granulocytes and thin-walled blood vessels. This tumor is positive for vimentin and CD-34 and focally expressing SMA and negative for anaplastic lymphoma kinase (ALK) and b-catenin.^[13]

Gastric schwannoma is a benign tumor that occurs commonly in the elderly, with a variable-sized mass of 2–5 cm. Schwannoma has clinical manifestations similar to those of GIST. The tumor is composed of spindle-shaped cells, arranged in criss-crossing bundles with variable amounts of collagen between cells. The nuclei have “picket fence” arrangement. The tumor is surrounded by lymphocytic nests. Tumor cells express nerve markers such as S-100, Leu-7, PGP9.5 and GFAP. Some tumors also express CD34. Schwannomas are negative for CD117, DOG1, ALK, desmin and SMA.^[14]

Gastric leiomyoma is not common as oesophageal or colonic myoma. Tumors size may reach a diameter >5 cm. It is composed of well-differentiated smooth muscle cells, with no atypia. The tumor IHC is positive for desmin and SMA and negative for CD117, ALK and CD34.^[15]

Inflammatory myofibroblastic tumors are most commonly occur in children <10 years of age, histologically composed mainly of proliferating obese spindle-shaped fibroblasts or myofibroblasts, with inflammatory cells such as mature plasma cells, lymphocytes and eosinophils and neutrophils. The immune stains are positive for vimentin and SMA, and desmin and MSA are focally positive. Fifty per cent of the cases are positive for ALK, and it stains negative for CD117. DOG1, β-catenin and S-100 protein.^[16]

Myofibromas/myofibromatoses are rare benign tumors formed of muscle-like cells with the contractile element. It can occur from newborns to the elderly, but many cases are seen in infants and children <2 years of age. It affects internal organs in 15%–20%, such as lung, heart, gastrointestinal tract, liver, kidney and pancreas. Histologically, the tumor cells are spindle shaped, with pale pink cytoplasm and long spindle-shaped nucleus. Immunophenotyping is positive for vimentin, SMA and whole actin HHF35 and negative for CK, EMA, ALK, β-catenin and S-100 protein.^[17]

Gastric solitary fibrous tumor is formed of irregularly distributed tumor cells with dense collagen fiber deposition and haemangioma-like regions. Moderate-to-severe atypia with area of necrosis may be present. Lymph node metastasis is possible. The tumor stains are positive for CD99, CD34, Bcl-2 and vimentin. CK pan, EMA, SMA, S-100 protein and desmin. It stains negative for ALK, CD68, CD163, CD21, CD23, β-catenin, CD117 and DOG1.^[18]

Scirrhous carcinoma of the stomach is commonly seen in the 30–40 years' age group. It presents with anorexia and weight loss. The tumor cells are small with fibrous connective tissue. Sometimes, the tumor may contain spindle-shaped or irregular-shaped cells. The tumor has frequent mitotic figures with lymphovascular invasion. The tumor is positive for CK pan, CK8/18, CK19, CK20, EMA and CEA and negative for vimentin, SMA, desmin and S-100 protein.^[19]

Surgical resection with negative margins is the cornerstone for the treatment of gastric fibromatosis. Several treatment options, including radiotherapy, hormone therapy, non-steroidal anti-inflammatory drugs and observation, have been proposed, and these treatment modalities vary depending on the involvement of vital structures.^[20] Strict follow-up is ensured for this patient in our study in the next 5 years due to the large size of the tumor >5 cm, and she did not receive any adjuvant therapy.

Conclusion

Gastric fibromatosis is a rare locally invasive mesenchymal tumor. It mimics other gastric mesenchymal tumors such as GIST. It is diagnosed microscopically with immunohistochemistry. Surgical resection is the main line of treatment. Close follow-up is necessary after surgery due to the high recurrence rate.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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