Tratamiento Conservador del Fibroma Desmoplásico Maxilar en Paciente Pediátrico. Seguimiento a Dos Años: Reporte de Caso y Revisión de la Literatura

RESUMEN: El fibroma desmoplasico (FD) es una neoplasia intraósea benigna, localmente agresiva, de muy baja frecuencia - especialmente en niños pequeños - y es considerada la contraparte ósea de la fibromatosis tipo desmoide. Se han descrito tratamientos que van desde la enucleación y legrado hasta la resección del segmento óseo afectado, debido a su alta tasa de recidiva. Sin embargo, se ha establecido un consenso para evitar dichas recidivas, que consiste en realizar una resección segmentaria con márgenes de seguridad. En este reporte de caso se decidió realizar un tratamiento conservador debido a la edad de la paciente, siendo el caso más joven reportado en la literatura con un FD maxilar y, de esta manera, evitar mutilaciones y necesidad de reconstrucción ósea a tan corta edad, considerando también su estado de crecimiento, generación de potenciales asimetrías o desarrollo facial anormal que podría generar secuelas estéticas y funcionales, alterando su calidad de vida.

ABSTRACT: Desmoplastic fibroma (DF) is a benign, locally aggressive, intraosseous neoplasm of very low frequency - especially in young children - and is considered the bony counterpart of desmoid-type fibromatosis. Treatments ranging from enucleation and curettage to resection of the affected bone segment have been described, due to its high recurrence rate. However, a consensus has been established to avoid such recurrences, which consists in performing a segmental resection with safety margins. In this case report, it was decided to carry out a conservative treatment due to the age of the patient, being the youngest case reported in the literature with a maxillary DF and, in this way, avoiding mutilations and the need for bone reconstruction at such a young age. Also considering their growth status, generation of potential asymmetries or abnormal facial development that could generate aesthetic and functional sequelae, altering their quality of life.

Clinical analysis of 8 cases of desmoplastic fibroma of the jaw / 口腔疾病防治

Objective @#To improve the dentist's understanding of desmoplastic fibroma of the jaw, we investigated the clinical manifestations, pathological features, treatment and prognosis of this disease.@*Methods@#The clinical data of 8 patients with desmoplastic fibroma of the jaw who were admitted to Nanjing Stomatological Hospital from 2011 to 2021 were retrospectively reviewed.@* Results @#The male-female ratio in this group was 3:1, the age of first onset was 32.13±15.00, and the lesions were mainly in the mandible. Histologically, the lesions was composed of mildly atypical fibroblasts and a large number of collagen fibers. The positive rates of Vimentin, α-SMA and β-catenin in the cytoplasm were 100%, 62.5% and 62.5%, respectively. The Ki-67 level in the initial patients was lower than 5%, and the S-100 protein level was 100% negative. The imaging manifestations were single-room or multichamber light-transmitting lesions with clear or irregular boundaries, with or without peripheral sclerosis. Five patients were treated with curettage for the first time; among them, two patients relapsed with poor prognosis. Three patients underwent extended resection, and all had no recurrence.@*Conclusions @# The clinical and imaging features of desmoplastic fibroma of the jaw are not specific. We mainly rely on histopathology to diagnose the disease. It has a high recurrence rate after surgery. At present, the best treatment is to extend surgical resection. Local curettage is easy to relapse and has a poor prognosis.

Desmoid Fibromatosis A Rare Case Report

Desmoidfibromatosisis a rare tumor affectingbone.This tumorhistologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. This is locally infiltrative and aggressive in nature. The reported incidence ofsuch cases is around 2–4 per million population whichaccountsfor 0.03% of all neoplasms. In maxillofacial region, incidence is less than 3% of all cases. Treatment of such tumors is surgical excision and chances of recurrence are more. Weherebyreport a case of a 23-year-old female patient withdesmoid fibromatosis in the mandibular posterior region.

Desmoplastic fibroma of the bone: a clinicopathological analysis of seven cases / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic features of desmoplastic fibroma of bone (DF).Methods The clinical and pathologic profiles of 7 cases were retrospectively analyzed.Results Desmoplastic fibroma of bone was a rare neoplasm of bone,which typically occurred in young children and adolescents.The tumor showed infiltrative and locally aggressive nature,the most common sites were long tubular bones and mandible.Microscopically,the tumor was composed of sparse fibroblasts in a rich background of collagen fibers.Immunohistochemically,the tumor cells were positive for vimentin and SMA.Conclusion Desmoplastic fibroma is a rare,locally aggressive fibroblasticlesion of bone.It can make an accurate diagnosis by integrating the clinical location with histopathological features.Trauma,hormone and horomosome aberration may be related with its pathogenesis.

Fibroma desmoplásico en la cadera. Presentación de un caso / Desmoplastic fibroma in the hip. Case presentation

Se presentó un caso de un fibroma desmoplásico en la cadera en un paciente de 28 años de edad. Se realizó estudio radiográfico que reveló lesión osteolítica extensa en cadera derecha. El objetivo fue mostrar una tumoración con poca frecuencia estadística en la bibliografía nacional e internacional y se expuso el proceder y tratamiento definitivo. El estudio histológico corroboró presencia de abundantes fibras colágenas, que formaban bandas hialinizadas, separadas por escasos fibroblastos de tamaño pequeño, sin actividad mitótica. Se realizó curetaje de la lesión con injerto de Hidroxiapatita porosa HAP-200. El paciente tuvo una evolución postquirúrgica satisfactoria con incorporación inmediata al medio social.

We presented a case of a desmoplastic fibroma in the hip in a patient aged 24 years. A radiographic study was carried out revealing an extensive osteolytic lesion in right hip. The aim was showing a tumor with little statistical frequency in national and international literature, and exposing the procedure and definitive treatment. The histological study confirmed the presence of abundant collagenic fibers, forming hyaline bands separated by few little fibroblasts, without mitotic activity. We made the curettage of the lesion with a graft of porous hydroxyapatite HAP-200. The patient had a satisfactory post-surgical evolution with an immediate incorporation to the social environment.

Large desmoplastic fibroma: case report using microvascularized fibula graft for reconstruction / Fibroma desmoplásico de grandes proporções: relato de caso usando enxerto microvascularizado de fibula para reconstrução

The Desmoplastic Fibroma is a benign rare tumor of fibroblastic origin, representing only 0.03% of benign bone tumors. Displays aggressive behavior and high rates of recurrence, which makes the treatment more aggressive. The authors present a case of an 11 years-old female, complaining of increased volume in the jaw. The patient had an extensive swelling of the left mandibular body with invasion of the border of the tongue and floor of the mouth at the same side. She had a history of 4 recurrences at the same site. Image exams revealed expansive lesion in body and ramus of the mandible with involvement of soft tissue on the lingual side of the lesion. The treatment was excision of the lesion with extra oral access and reconstruction with microvascularized fibula graft. The patient has been followed for 2 years with no clinical or radiographic signs and without recurrence

O Fibroma Desmoplásico é um tumor benigno raro de origem fibroblástica que representa apenas 0,03% dos tumores ósseos benignos. Apresenta comportamento agressivo e elevadas taxas de recorrência, o que torna o seu tratamento mais agressivo. Os autores apresentam um relato de caso de uma paciente do sexo feminino de 11 anos de idade com queixa de aumento de volume em mandíbula. A mesma apresentou extensa expansão do corpo mandibular esquerdo, com invasão da borda da língua e assoalho da boca do mesmo lado, com histórico de quatro recorrências na mesma localização. Os exames de imagem revelaram lesão expansiva no corpo e ramo da mandíbula com envolvimento dos tecidos moles do lado lingual da lesão. O tratamento foi a excisão da lesão com acesso extra-oral e reconstrução com enxerto microvascularizado de fíbula. A paciente apresenta seguimento de 2 anos sem sinais clínicos ou radiográficos de recidiva.

Pediatric fibromatosis involving mandible: case reportand a five-year post-operative follow-up / Fibromatose pediátrica em mandíbula: relato de caso e avaliação pós-operatória de cinco anos

Extra-abdominal fibromatosis is a benign fibrous neoplasm of locally aggressive behavior. Surgical excision with a wide margin is the treatment of choice. The aim of the present work is to report the case of fibromatosis in an 11-year-old melanodermic patient, who showed swelling in the area of the right mandible. Conservative surgery was performed based on the initial histopathological diagnosis of benign lesion suggestive of neural origin. The new anatomopathological examination of the surgical specimen was compatible with fibromatosis. The patient is still under periodic observation as part of the five-year surgical follow-up, showing no signs of recurrence.

Fibromatose extra-abdominal é uma neoplasia fibrosa benigna, caracterizada por comportamento agressivo local. Geralmente, o tratamento de escolha consiste na excisão cirúrgica com ampla margem de segurança. O objetivo deste trabalho é relatar um caso de fibromatose em uma paciente de 11 anos, melanoderma, que apresenta aumento de volume na mandíbula do lado direito. A partir do diagnóstico histopatológico inicial sugestivo de lesão benigna de origem neural, foi realizado tratamento cirúrgico conservador. Um novo exame anatomopatológico da peça cirúrgica foi compatível com fibromatose. A paciente encontra-se sob controle periódico de cinco anos da cirurgia, não havendo recorrência clínica da lesão.

Desmoplastic Fibroma of Bone in a Toe: Radiographic and MRI Findings

Desmoplastic fibroma is a rare benign primary bone tumor that is histologically similar to the soft tissue desmoid tumor. It most often involves the mandible, large long bone or iliac bone. Desmoplastic fibroma in a toe has been extremely rarely reported. Authors report a rare case of desmoplastic fibroma of bone occurring in the distal phalanx of a foot, with descriptions of the radiographic and MRI findings, correlation of the radiologic and pathologic findings, and discussion on the differential diagnosis of the tumor.

Fibroma desmoplásico de la columna lumbar / Desmoplastic fibroma of lumbar spine

Introducción: el fibroma desmoplásico fue definido por la Organización Mundial de la Salud en 2002 como un tumor benigno, raro, compuesto por células fusiformes, con mínima atipia celular y abundante producción de colágeno. Es considerado por muchos autores como benignos localmente agresivos, pero que rara vez hacen metástasis. Objetivo: presentar un caso raro de fibroma desmoplásico en la columna lumbar y discutir el diagnóstico y tratamiento de esta afección. Descripción: se presentó un paciente de 20 años de edad con cuadro doloroso en la columna lumbar. Se hizo una radiografía simple observándose imagen osteolíotica de la apófisis espinosa de L3, se decidió efectuar biopsia abierta incisional que determinó un fibroma desmoplásico óseo. Se aplicó tratamiento quirúrgico con bordes amplios de la lesión, mediante abordaje combinado anterior y posterior. La reconstrucción se realizó con tornillos pediculares, placa anterior e injerto autólogo de peroné. Resultados: después de la cirugía el dolor desapareció y la función neurológica resultó normal. Hubo complicaciones relacionadas con el injerto óseo e infección del sitio quirúrgico. El paciente en un seguimiento de 3 meses no tuvo recidiva. Conclusión: el fibroma desmoplásico óseo, descrito también como quiste desmoide óseo, es un tumor muy infrecuente y de difícil diagnóstico, el cual debe ser considerado entre los diagnósticos diferenciales de los tumores de la columna vertebral. Una confirmación histológica siempre es requerida. La resección en bloque de la lesión puede ser curativa seguida de la reconstrucción para preservar la estabilidad espinal

Introduction: the desmoplastic fibroma was defined by WHO in 2000 as a benign tumor, rare, composed by fusiform cells with a minimal cellular atypia and an abundant collagen production. Many authors consider it as locally aggressive and benign tumors but rarely to metastasize. Objective: to present a rare case of desmoplastic fibroma in lumbar spine and to discuss the diagnosis and treatment of this affection. Description: this is the case of a patient aged 20 presenting with a painful picture in lumbar spine. A simple radiography was taken showing an osteolytic image of spinal apophysis of L3 and an open incisional biopsy determining a bone desmoplastic fibroma. Surgical treatment was applied with wide edges of injury by anterior and posterior combined approach. In repair process we used pedicular screws, anterior plate and autologous peroneal graft. Results: after surgery pain disappeared and the neurologic function became normal. There were complications related to the bone graft and infection in surgical site. During a 3-month follow-up patient had not relapse. Conclusion: the bone desmoplastic fibroma also known as bone desmoid cyst is a very infrequent tumor and of difficult diagnosis, which must to be considered among the differential diagnoses of spinal column tumors. Always it is necessary a histological confirmation. The block resection of injury must to be curative followed by the repair to preserve the spinal stability

Desmoplastic Fibroma of the Cranium in a Young Man

Desmoplastic fibroma, which develops predominantly in long bones and the mandible, is a rare and benign but locally aggressive tumor. Desmoplastic fibroma of the cranium is extremely rare. We report a case of desmoplastic fibroma of the frontal bone in a young man. Because of its locally aggressive behavior, complete surgical excision with a safety margin is essential.

Fibroma desmoplásico del hueso / Bone desmoplastic fibroma

Se presentó un caso de un fibroma desmoplásico en una adolescente de 14 años de edad. Se realizó estudio radiográfico que reveló lesión osteolítica diafisometafisaria extensa en fémur derecho. El objetivo fue mostrar una tumoración con poca frecuencia estadística en la bibliografía nacional e internacional y mostrar el proceder y tratamiento definitivo. El estudio histológico corroboró presencia de abundantes fibras colágenas, que formaban bandas hialinizadas separadas por escasos fibroblastos de tamaño pequeño sin actividad mitótica. Se realizó curetaje de la lesión con inserción de cemento quirúrgico. La paciente tuvo una evolución posquirúrgica satisfactoria con incorporación inmediata al medio social

This is the case of a desmoplastic fibroma present in an adolescent aged 14. A radiographic study was conducted showing an extensive osteolytic diaphysis and metaphyseal lesion in right femur. The objective of present paper was to present a tumor with little statistic frequency in the national and international bibliography and also to show the procedure and definitive treatment. The histological study corroborates the presence of abundant collagen fibers forming hyalinization strips separated by scarce small fibroblasts without mitosis activity. Curettage of lesion was performed with insertion of surgical cement. Patient had a satisfactory postsurgical course with an immediate incorporation to social environment

Desmoplastic Fibroma of Distal Femur: A Case Report

Desmoplastic fibroma of bone is an extremely rare tumor that was first described by Jaffe in 1958. It histologically resembles the desmoid tumor of soft tissue. It is known as locally aggressive tumor but we experienced definitely benign and resembling simple bone cyst radiographically. We report a case of desmoplastic fibroma of bone and it should be included in the differential diagnosis list of any lytic bone lesion. The radiograph, MR imaging features, radiological and pathological differential diagnosis of the case are described, and literatures are reviewed.

Desmoplastic Fibroma of the Skull

Desmoplastic fibroma is one of the uncommon osseous tumors that present in the skull. Although classified as benign tumor, desmoplastic fibroma exhibits local aggressiveness and has a high potential for recurrence. The most common sites include metaphysis of long bones and mandible. Only 15cases have been described in the skull. We report the 16th case of desmoplastic fibroma of the skull.

Fibroma desmoplásico mandibular / Mandibular desmoplastic fibroma

Se presenta un caso de fibroma desmoplásico en una niña de 3 años de edad con historia de aumento de volumen de 6 meses de evolución en zona de hueso mandibular del lado derecho. Fue tratada quirúrgicamente bajo anestesia general, realizando la resección radical y la reconstrucción mandibular simultáneamente. Este reporte revisa criterios de diagnóstico y manejo quirúrgico de este tumor benigno poco común que afecta a la región maxilofacial.

This is the case of a desmoplastic fibroma affecting the jaw of a 3 year old girl with history of volume increase of 6 month­evolution in the right mandibular bone. The lesion was surgically treated under general anesthesia, performing the radical resection and the mandibular reconstruction simultaneously. This report reviews the diagnostic criteria and surgical management of this uncommon benign tumor affecting the maxillofacial region.

Desmoplastic Fibroma of the Skull

Desmoplastic fibromas are rare intraosseous bone tumors. They are benign but locally aggressive and frequently found in the long bones and mandible. We report radiographic and histopathologic finding of a case desmoplastic fibroma involving right temporal skull bone. A 53-year-old woman presented at our hospital complaining of continuous right side headaches for a year. Simple skull X-ray film showed 3 x 2.5 cm lytic lesion with mild sclerotic margin on right temporal area. A large craniectomy 1cm lateral to margin was fashioned. The resected mass showed encapsulated mass colored white gray. Histologic diagnosis was compatible with that of a the desmoplastic fibroma. There was no evidence of recurrence during the 15months of follow-up period.

Desmoplastic Fibroma of the Skull;A Case Report, Review of the Literature, and Therapeutic Implications

Desmoplastic fibroma(DF) is a rare neoplasm of the bone, and is histologically benign but locally aggressive disease. A total of nine cases of DF involving skull have been reported in the literature, and among these eight are females. In this report, the clinical findings and histopathology of a case with DF of the skull in a male patient is presented with a review of the literature with an emphasis on treatment modalities. A 21-year-old man presented with headache. CT scan revealed a solitary and lytic skull lesion without brain invasion. DF was confirmed by histological evaluation. On immunohistochemical staining of the tumor was negative for estrogen or progesterone receptors. After total resection of tumor with wide surgical margin, there was no recurrence during the 35 months of follow-up period. Although longer follow up period maybe needed, treatment of this type of tumor with complete resection of tumor tissue along with a wide margin may provide long disease-free state compare to the high recurrence rates in DF of other sites.

Desmoplastic Fibroma Of The Mandible / 대한구강악안면방사선학회지

Desmoplastic fibroma is a rare, benign intraosseous fibroblastic tumor, which is locally aggressive. It is osseous counterpart of soft tissue fibromatosis. The authors experienced the patient who complained persistent mouth opening limitation with mild swelling on the left mandibular angle area. After careful analysis of clinical, radiological and histopathological findings, we diagnosed as desmoplastic fibroma of the mandible. The results were as follows: 1. Main clinical symptoms were mouth opening limitation which had been persistent for 9 months and mild swelling on the left mandibular angle area. 2. Radiographs showed the radiolucent lesion and expansion of lingual cortex. CT finding is homogeneous soft tissue mass with expansion of left mandibularramus. Destruction of medial wall of ramus and invasion to adjacent soft tissue is also seen. 3. Histopathologically, plump spindle shaped fibroblasts arranged in bundles or fascicles are observed. The cells of tumor are infiltrating into muscle fiber with destruction of bony trabeculae and merged with surrounding salivary gland.

Desmoplastic Fibroma Of The Mandible / 대한구강악안면방사선학회지

Desmoplastic fibroma is a rare, benign intraosseous fibroblastic tumor, which is locally aggressive. It is osseous counterpart of soft tissue fibromatosis. The authors experienced the patient who complained persistent mouth opening limitation with mild swelling on the left mandibular angle area. After careful analysis of clinical, radiological and histopathological findings, we diagnosed as desmoplastic fibroma of the mandible. The results were as follows: 1. Main clinical symptoms were mouth opening limitation which had been persistent for 9 months and mild swelling on the left mandibular angle area. 2. Radiographs showed the radiolucent lesion and expansion of lingual cortex. CT finding is homogeneous soft tissue mass with expansion of left mandibularramus. Destruction of medial wall of ramus and invasion to adjacent soft tissue is also seen. 3. Histopathologically, plump spindle shaped fibroblasts arranged in bundles or fascicles are observed. The cells of tumor are infiltrating into muscle fiber with destruction of bony trabeculae and merged with surrounding salivary gland.

A Case of Desmoplastic Fibroma of the Mandible

A case of desmoplastic fibroma of mandible in a 18 years old woman is presented. She had complained progressive swelling of right mandible for 4 years. Radiographically, a multilocular radiolucent of right hemimandibulectomy showed multinodular external surface without cortical destruction. Cut surfaces revealed grayish white, fibrous homogeneous appearance with firm consistency, sparing head portion. The maximum diameter of the tumor was 13 cm. Microscopically, the tumor was composed of interlacing bundles of monomorphic spindle-shaped cells with abundant intercellular collagen. Ultrastructurally, most tumor cells were fibroblastic-like cells with abundant RER and cytoplasmic fibrils, but a few disclosed transition to myofibroblasts. However, no fully developed myofibroblasts were seen.

A Case of Desmoplastic Fibroma of the Mandible

A case of desmoplastic fibroma of mandible in a 18 years old woman is presented. She had complained progressive swelling of right mandible for 4 years. Radiographically, a multilocular radiolucent of right hemimandibulectomy showed multinodular external surface without cortical destruction. Cut surfaces revealed grayish white, fibrous homogeneous appearance with firm consistency, sparing head portion. The maximum diameter of the tumor was 13 cm. Microscopically, the tumor was composed of interlacing bundles of monomorphic spindle-shaped cells with abundant intercellular collagen. Ultrastructurally, most tumor cells were fibroblastic-like cells with abundant RER and cytoplasmic fibrils, but a few disclosed transition to myofibroblasts. However, no fully developed myofibroblasts were seen.