Antral plexiform fibromyxoma: case report of a rare mesenchymal neoplasm

Plexiform fibromyxoma (PF) is a rare mesenchymal neoplasm of the stomach usually arising in the gastric antrum, and its main differential diagnosis is gastrointestinal stromal tumor. Most common symptoms are hematemesis, anemia. Immunohistochemically, positivity for smooth muscle actin (SMA) and vimentin suggests the diagnosis of PF. We report the case of a 56-year-old female patient with a 30- day history of nausea at presentation 4 years ago. Gastroscopy at that time revealed a subepithelial lesion (SEL) in the gastric antrum, measuring approximately 20 mm in diameter, with leakage of serous fluid after biopsy. Histopathology showed only an inflammatory process. Follow-up gastroscopies were performed 24, 36, and 48 months later, with surveillance biopsy at each follow-up. The last gastroscopies showed changes in lesion appearance, reduction in size, and absence of fluid leakage. Histopathology showed bland spindle cell proliferation, with a vaguely plexiform/multinodular pattern, in a fibromyxoid stroma with an arborizing capillary network without mitoses. The tumor cells were positive for SMA and negative for DOG1, CD117, CD34, S100, desmin, EMA, CD10, calponin, and beta-catenin. The choice of treatment and follow-up depends on the SEL features, but because no cases of malignancy or metastatic disease have previously been reported, the patient chose a conservative approach.

El fibromixoma plexiforme (FP) es una rara neoplasia mesenquimatosa del estómago que generalmente surge en el antro gástrico. Su principal diagnóstico diferencial es el tumor del estroma gastrointestinal. Los síntomas más comunes de los FP son hematemesis y anemia. Inmunohistoquímicamente, la positividad para actina del músculo liso (SMA) y vimentina sugieren el diagnóstico de FP. Presentamos el caso de una paciente de 56 años de edad que inicia su enfermedad hace 4 años con náuseas de 30 días de evolución. La primera gastroscopia reveló una lesión subepitelial (SEL) en el antro gástrico, de aproximadamente 20 mm de diámetro, con fuga de líquido seroso después de la biopsia. La histopatología mostró sólo un proceso inflamatorio. Se realizaron gastroscopias de seguimiento a los 24, 36 y 48 meses con biopsia de vigilancia en cada seguimiento. Las gastroscopias siguientes mostraron cambios en la apariencia de la lesión, reducción de tamaño y ausencia de fuga de líquido. La última histopatología mostró una proliferación blanda de células fusiformes, con un patrón vagamente plexiforme/multinodular, en un estroma fibromixoide con una red de capilares arborizantes sin mitosis. Las células tumorales fueron positivas para SMA y negativas para DOG1, CD117, CD34, S100, desmina, EMA, CD10, calponina y beta-catenina. La elección del tratamiento y el seguimiento depende de las características del SEL, sin embargo, por ser una enfermedad que no presentaba rasgos de enfermedad maligna o metastásica, el paciente eligió un mantener un enfoque conservador.

Plexiform fibromyxoma of the stomach: An underrecognized entity!

Plexiform fibromyxoma (PF) is a recently described rare type of mesenchymal tumor of the stomach with only 123 cases reported in the literature. It is characterized by a peculiar plexiform growth pattern, myxoid stroma with arborizing microvasculature, and spindle-shaped myofibroblastic cells. We herein report a case of gastric PF in a 15-year-old boy, mimicking a gastrointestinal stromal tumor (GIST) due to overlapping clinicoradiological features. Distinct pathological and immunohistochemical features of PF do aid in distinction from GIST and other mesenchymal entities. Diagnosis is crucial as surgical resection is the mainstay of treatment unlike aggressive management in GIST. It is a benign entity with no local recurrence or distant metastasis reported so far, but confirmation of the same requires longitudinal observational studies with a larger sample size.

Fibromixoma Odontogénico Agresivo en Maxila: Abordaje Quirúrgico / Aggressive Odontogenic Fibromixoma in Maxilla: Surgical Approach

RESUMEN: El fibromixoma odontogénico (FM) es una infrecuente lesión benigna que muestra un comportamiento invasivo. Considerada una variante del mixoma odontogénico, presenta un origen mesenquimal controvertido y ligera predilección por el sexo femenino. La lesión está asociada a zonas con estructuras dentales adyacentes, siendo su localización más frecuente la región mandibular posterior. El objetivo de este trabajo fue mostrar un caso agresivo de FM en una paciente, localizado en la zona posterior de maxila, destacando su abordaje quirúrgico. Se reporta un caso de una mujer de 52 años, la cual acudió a la consulta por presentar una masa indolora, firme a la palpación en el lado derecho de la maxila. Radiográficamente se observaba como una imagen radiopaca, de márgenes mal definidos que se extendía en el seno maxilar ipsilateral; la tomografía computarizada pudo evidenciar la expansión de las paredes del antro y la fosa nasal, con compromiso del cigomático y el piso de órbita. Se realizó biopsia incisional y el diagnóstico histopatológico fue de FM. El tratamiento de elección consistió en la resección en bloque, mediante abordaje Weber-Fergusson, acompañada de reconstrucción con placa y malla de titanio para garantizar la suspensión del globo ocular; un nuevo estudio histopatológico, confirmó el diagnóstico inicial. Al año del procedimiento quirúrgico no se evidenció recidiva, observando que el material de reconstrucción mantiene su posición, proporcionado estética y funcionabilidad a la paciente.

ABSTRACT: Odontogenic fibromyxoma (FM) is an infrequent benign lesion that shows with invasive characteristics. Considered a variant of the odontogenic myxoma, it presents a controversial mesenchymal origin and is somewhat more frequent in women. The lesion is associated with areas with adjacent dental structures, with its most frequent location in the posterior mandibular region. The objective of this study was to show an aggressive case of FM in a female, located in the posterior area of the maxilla, emphasizing its surgical approach. A case of a 52-year-old woman, who came to the clinic because of a painless, firm mass on the right side of the maxilla. Radiographically, it was seen as a radiopaque image, with poorly defined margins extending in the ipsilateral maxillary sinus; computed tomography showed the expansion of the walls of the antrum and the nasal cavity, with zygomatic compromise and the orbital floor. An incisional biopsy was performed and the histopathological diagnosis was FM. The treatment of choice consisted of block resection, using a WeberFerguson approach, along with reconstruction with a titanium plaque and mesh to guarantee suspension of the eyeball; a new histopathological study, confirmed the initial diagnosis. One year after the surgical procedure, there is no recurrence, and the reconstruction material maintains its position, providing aesthetic and functionality to the patient.

A Case of Fibromyxoma Mistaken as an Antrochoanal Polyp

Fibromyxomas are uncommon, tenaciously infiltrative neoplasms that infrequently appear in the facial bones and paranasal sinuses. The neoplasms are slow growing and result in expansion of the surrounding bony cortices. In the present study, we report an extremely rare case of antrochoanal fibromyxoma that occluded the ostiomeatal complex and originated from the maxillary sinus inferior wall. Initially, the neoplasm was suspected to be a nasal polyp. However, after endoscopic sinus surgery, the neoplasm was diagnosed as fibromyxoma based on histopathology.

A Case of Superficial Acral Fibromyxoma Showing Erythronychia / 대한피부과학회지

No abstract available.

A Case of Fibromyxoma of the Maxilla

Fibromyxoma is a rare mesenchymal tumor that is benign, but locally invasive. It is a slow-glowing painless tumor with the potential for extensile bony destruction and cortical expansion and shows a relatively high recurrence rate. Fibromyxoma is found predominantly in the jaw, with the mandible more frequently affected than the maxilla. We recently experienced a case of fibromyxoma originating from the maxilla in a 50-year-old woman who complained of swelling on the right side of her cheek. En bloc resection via a sublabial approach and middle meatal antrostomy were performed. A diagnosis of fibromyxoma was based on pathologic findings. No recurrence or locally residual lesion has been found during 2-years follow up. Therefore, we present this rare case with a review of the literature.

Fibromixoma de septum nasal: Presentación de un caso / Nasal septum fibromyxoma: A case report

Introducción: El fibromixoma es una lesión benigna poco frecuente cuya etiología aún es controversial. Rara vez aparece en cabeza y cuello, en cuyo caso afecta con mayor frecuencia a la mandíbula. Objetivo: Presentar un caso poco frecuente de fibromixoma derivado de septum nasal. Material y métodos: A continuación se describe el caso de un paciente masculino de 50 años con diagnóstico de fibromixoma proveniente del septum nasal, que se presento con epífora como única manifestación clínica; se trató quirúrgicamente con resección total endoscópica. Discusión: Son pocos los casos de fibromixoma documentados que comprometen la región nasosinusal. Conclusiones: Es importante considerar al fibromixoma dentro de los tumores del septum nasal, que a pesar de su naturaleza benigna, son lesiones que requieren resecciones amplias por su comportamiento localmente agresivo.

Introduction: Fibromyxoma is an uncommon benign lesion whose etiology is still controversial. Rarely, it appears in head and neck where the jaw is the region most often affected. Objective: To present a rare case of nasal septum fibromyxoma. Methods: We present the case of a 50 year old male with the diagnosis of fibromyxoma from the nasal septum, who presented with epiphora as the only clinical manifestation. It was treated surgically with complete endoscopic resection. Discussion: There are few cases of fibromyxoma documented that compromise sinonasal region. Conclusions: It is important to consider fibromyxoma within the differential diagnosis of nasal septum tumors, which despite its benign nature, requires extensive resections due to its locally aggressive behavior.

Paediatric Fibromyxoma Maxilla- a rare entity.

Fibromyxoma is a rare benign tumour of mesenchymal origin that mostly involves the posterior part of the mandible. It is a locally aggressive and slowly growing painless tumour that mostly occurs in second and third decades of life. We report a case of 2 years old child with huge mass of the right maxilla. After proper diagnosis mass was completely excised via sublabial approach and reported histopathologically as fibromyxoma. Because of its rarity in the maxilla and in this age, it is being reported here.

Radiographic Presentation of Fibromyxoma: An Uncommon Entity.

Fibromyxoma is a rare, locally destructive, benign odontogenic mesenchymal tumor which is found exclusively in the jaw region. The maxilla and anterior part of the mandible are rarely affected. It can usually lead to extensive bony destruction with extension into the surrounding structures. The lesion often grows without symptoms and presents as a painless swelling. Histologically and radiologically it is difficult to differentiate this tumor from other odontogenic tumors and may be mistaken occasionally for a malignant pathology. A similar situation happened with our case that was clinically misdiagnosed as malignant lesion initially, but after two repeated biopsies diagnosed as a fibromyxoma.

Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach: A Case Report

Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described mesenchymal tumor of the stomach. We report the first case of PAMT in Korea. A 52-yr-old man underwent esophagogastroduodenoscopy due to dyspepsia for 2 yr. There was a submucosal mass with small mucosal ulceration in the gastric antrum. The tumor measured 3.5 x 2.3 cm in size and showed multinodular plexiform growth pattern of bland-looking spindle cells separated by an abundant myxoid or fibromyxoid matrix rich in small thin-walled blood vessels. The tumor cells were negative for CD117 (c-KIT), CD34 and S-100 protein, but diffusely positive for smooth muscle actin consistent with predominant myofibroblastic differentiation. The patient is doing well without recurrence or metastasis for 5 months after surgery. Although there have been limited follow-up data, PAMT is regarded as a benign gastric neoplasm with histological and immunohistochemical charateristics distinguished from gastrointestinal stromal tumor and other mesenchymal tumors of the stomach.

Fibromixoma odontogénico y xantoastrocitoma en paciente adolescente: ¿asociación clínica? Reporte de un caso y revisión de la literatura

El fibromixoma odontogénico es un tumor odontogénico derivado del ectomesénquima, de naturaleza benigna pero localmente invasivo. Se reporta el caso de una joven paciente con un fibromixoma odontogénico en el maxilar superior, con antecedente de un Xantoastrocitoma en el hemisferio derecho, extirpado dos años antes de la aparición del tumor del maxilar. Se realizó un tratamiento conservador por ser una lesión pequeña limitada al sector anterior del maxilar con la estrecha observación clínica postoperatoria.

Odontogenic fibromyxoma is an odontogenic tumor derived from ectomesenchime, benign but locally aggressive. A case of a young female patient with an odontogenic fibromyxoma in upper jaw is reported, she had a Xantoastrocitoma in the right hemisphere which was removed a year before the appearance of the tumor on the maxilla. Due to the size of the lesion a conservative surgical approach was made as treatment. Followed by a close clinical follow up.

A Case of Recurrent Superficial Acral Fibromyxoma

Superficial acral fibromyxoma (SAFM) is a rare myxoid tumor that was first described in 2001. The presence of a very slow growing solitary tender mass in the subungual area is the typical clinical feature at presentation. Histopathologically, SAFM is composed of stellate cells in a myxocollagenous matrix with a poorly circumscribed margin. This tumor is thought to be benign, but its natural course is not fully understood. We describe a 15-year-old patient with recurrent SAFM and discuss the proper treatment and follow up.

A Case of Superficial Acral Fibromyxoma / 대한피부과학회지

Superficial acral fibromyxoma (SAF) is an uncommon soft tissue tumor that has recently been showed to be a separate disease entity. It is most often located in the ungal region of the fingers and toes of middle-aged adults. It is histologically characterized by a slight to moderate cellular proliferation of spindle cells and stellate cells arranged in a random, storiform or fascicular pattern, within a myxoid, myxocollagenous or collagenous stroma with a prominent vasculature. The neoplastic cells show positive staining for CD34, CD99 and EMA, but negative staining for S100, HMB45, cytokeratin, smooth muscle actin (SMA) and desmin. We report here on a typical case of SAF that occurred on the left index finger of a 44-year-old man.

A Case of Superficial Acral Fibromyxoma Occurring after Trauma in a Childhood Patient / 대한피부과학회지

Superficial acral fibromyxoma is a rare, distinctive soft tissue neoplasm most often located in the periungal region of the fingers and toes. This tumor was recently recognized as a distinct clinicopathological entity and since then quite a few cases have been reported. These tumors are composed of stellate and spindle cells arranged in a loose form, with a fascicular growth pattern, and occur mostly in middle-aged adults. However in our case, the lesion developed in 9-year-old male, who had a history trauma. Although the exact pathogenesis of superficial acral fibromyxoma has not yet been clarified, it is possible that the fibroblast hyperplasia in the lesion could be triggered by trauma. We suggest this case which occurred in a 9-year-old patient, triggered by trauma, is a peculiar and interesting event.

Odontogenic Fibromyxoma.

Odontogenic Fibromyxoma is a rare mesodermal tumour found exclusively in the bones of the facial skeleton. Although benign, it is locally very aggressive making it difficult to eradicate. We are reporting a case of odontogenic fibromyxoma in the maxilla with review of literature.

A Case of Ossifying Fibromyxoma of the Neck / 대한피부과학회지

An ossifying fibromyxoma is a benign neoplasm which usually presents in soft tissue. It is a rare tumor of uncertain differentiation which virtually always acts in a clinically-benign fashion. Most patients present with a small, painless, well-defined, often lobulated subcutaneous mass, which affects the extremities, or less commonly the trunk, head and neck, and mediastinum. Its typical microscopic appearance is that of lobulated nests of uniform, round-to-oval shaped cells, separated by fibromyxoid stroma, in which an incomplete marginal shell of mature bone can be seen. We herein report the first case of an ossifying fibromyxoid tumor of the neck in Korea.

Odontogenic fibromyxoma: A Case Report

Fibromyxoma Arising from the Maxilla: A Case Report / 대한이비인후과학회지

Fibromyxomatous lesions of the maxilla are rare. Although other locations are also uncommon, this tumor can occasionally be found in jawbone. They are slow-growing, which result in expansion of the surrounding bony cortices. Maxillary and man-dibular myxomas are locally aggressive neoplasms that are thought to arise from fibroblasts of the dental papilla or sinonasal mucosa. The precise origin of these tumors, however, remains controversial. When present in the maxilla, they can extend into the maxillary sinus, the nose, or the orbit. The differential diagnosis should be made from neoplasms in which myxomatous change can be a prominent secondary feature such as liposarcomas, chondrosarcomas, and neurofibromas. We report a case with a large maxillary mass occluding the nasal cavity and expanding hard palate and gingivobuccal area. The histopathologic diagnosis was odontogenic fibromyxoma originating from canine tooth. The treatment was medial maxillectomy.