Periosteal reaction as a crucial radiographic finding for desmoplastic fibroma of the jaw bone in children: A case report.

This report presents the case of a 5-year-old boy with a hard swelling on the right side of the mandible body. An important point of this case is that the primary imaging finding was fine spicules in the inferior border of the mandible on panoramic radiography without significant changes in bone density. Cone-beam computed tomography views revealed a lytic lesion on the lingual side of the right mandibular body with the destruction of the lingual cortex and periosteal reaction from the midline to the first molar area. Careful attention to this radiographic finding in the primary stage in the absence of other significant imaging findings, particularly in children, could result in the early diagnosis of desmoplastic fibroma. Therefore, a better prognosis can be expected following early surgical treatment.

Dural-Based Posterior Fossa Medulloblastoma Mimicking a Petrous Meningioma in Late Adulthood.

Background: Medulloblastoma of the posterior fossa is commonly encountered in pediatric populations but rarely reported in adults. Adult cases of medulloblastoma typically occur in younger patients, tend to arise intra-axially within the cerebellar hemisphere, and usually exhibit classic histopathologic features. Case Report: A 54-year-old male presented with headaches, dizziness, gait instability, and frequent falls that had worsened during the prior 3 months. Imaging and histopathologic analysis revealed extra-axial, dural-based posterior fossa medulloblastoma with desmoplastic/nodular histopathology, mimicking a petrous meningioma. The mass occupied the left cerebellopontine angle. The patient underwent microsurgical gross total resection of the tumor followed by proton beam radiation therapy and was disease-free at 1-year follow-up. Conclusion: Few dural-based posterior fossa medulloblastomas resembling petrous meningiomas have been reported, and to our knowledge, this is the first description of a case to be treated successfully with proton beam therapy in an older adult. Although rare, medulloblastoma can occur extra-axially in the cerebellopontine angle of older adults, potentially mimicking a petrous meningioma. This rare possibility should always be kept in mind, especially if expectant, nonsurgical management is being considered. To optimize outcome, posterior fossa medulloblastoma should be treated with aggressive microsurgical resection followed by radiation therapy. When available, proton beam therapy should be considered.

Fibromatosis tipo desmoide de apéndice cecal: reporte de caso inusual y revisión de la literatura / Appendicular desmoid tumor: unusual case and review of the literature

RESUMEN Antecedente : La fibromatosis tipo desmoide es un proceso neoplásico benigno no encapsulado localmente invasivo y agresivo, que se origina de la proliferación de fibroblastos y miofibroblastos aparentemente normales. La localización más frecuente de la fibromatosis es extra-abdominal (60%), pared abdominal (25%) e intra-abdominal (8-15%), en raras ocasiones puede originarse en las vísceras (0,73%), como el páncreas, unión gastroesofágica, diafragma y apéndice. La incidencia anual de tumor desmoide se estima de 2 a 5 casos por millón. En el presente artículo, reportamos un caso de presentación inusual, originado en el apéndice cecal. Caso : Paciente de sexo femenino de 41 años con dolor pélvico agudo, que ingresa a sala de operaciones con diagnóstico clínico y ecográfico de probable tumor de ovario a pedículo torcido. En el intraoperatorio se evidenció una tumoración sólida de 15 cm de diámetro que dependía del apéndice cecal, correspondiendo el estudio anatomopatológico a tumor desmoide de apéndice cecal. Conclusiones : El tumor desmoide puede originarse en diversas localizaciones extra e intra-abdominales, siendo esta última la más rara y agresiva. El diagnóstico preoperatorio exacto es muy difícil y casi siempre los pacientes ingresan al quirófano con sospecha diagnostica de tumoración intraabdominal de etiología desconocida. Los factores de riesgos asociados a su aparición aún no se encuentran caracterizados, siendo la resección quirúrgica completa del tumor -con márgenes quirúrgicos libres (R0)- el tratamiento de elección; sin embargo, el riesgo de recurrencia es alto incluso con la remoción óptima del tumor.

ABSTRACT Background : Desmoid type fibromatosis is a benign neoplastic process non-encapsulated locally invasive and aggressive, which arises from a proliferation of bland-looking fibroblasts and myofibroblasts. The most frequent location of fibromatosis is extra- abdominal (60%), abdominal wall (25%) and intra-abdominal (8-15%), rarely can originate in the viscera (0.73%), such as the pancreas, gastroesophageal junction, diaphragm and appendix. The annual incidence of desmoid tumor is estimated at 2 to 5 cases per million. In this article, we report a case of unusual presentation, originated in the cecal appendix. Case : A 41-year-old female patient with acute pelvic pain, admitted to surgical ward with a clinical and ultrasound diagnosis of probably ovarian pedicle tumor. An explorative laparotomy revealed a solid mass of 15 cm in diameter arising from the cecal appendix, with the anatomopathological study corresponding to a desmoid tumor of the cecal appendix. Conclusions : The desmoid tumor can arise from several extra and intra-abdominal locations, the latter being the rarest and most aggressive. Accurate preoperative diagnosis is very difficult and almost always patients enter to surgery with suspected diagnosis of intraabdominal tumor of unknown etiology. The risk factors associated to its appearance are not yet characterized. The complete surgical resection of the tumor -with free surgical margins (R0)- is the treatment of choice; however, the risk of recurrence is high even with optimal tumor removal.

Desmoplastic fibroma of the mandible: a rare gnathic bone tumor with a review of the literature

Desmoplastic fibroma (DF) is a rare bone tumor, which is known to involve mostly the gnathic bones. In this setting, the clinical presentation is usually represented by a bulging tumor of the face. Radiologically, the tumor is usually characterized by an expansile lytic bone lesion. The histopathology of the tumor shows a poorly circumscribed hypocellular lesion rich in collagen fibers with interspersed spindle cells having bland nuclear chromatin. Despite the lack of mitoses and nuclear pleomorphism, DF has an aggressive nature, presenting as a destructive growth causing entrapment of neuro-vascular bundles, sinusitis, or trismus. Some cases of DF show mutations in the adenomatous polyposis coli pathway shown by nuclear localization of the ß-catenin protein. Few reports showed an association with tuberous sclerosis, though most of these cases were sporadic. We discuss a rare case of desmoplastic fibroma involving the mandible, and a review of the literature of the DF cases involving the gnathic bones.

Desmoplastic fibroma of the mandible: a rare gnathic bone tumor with a review of the literature.

Desmoplastic fibroma (DF) is a rare bone tumor, which is known to involve mostly the gnathic bones. In this setting, the clinical presentation is usually represented by a bulging tumor of the face. Radiologically, the tumor is usually characterized by an expansile lytic bone lesion. The histopathology of the tumor shows a poorly circumscribed hypocellular lesion rich in collagen fibers with interspersed spindle cells having bland nuclear chromatin. Despite the lack of mitoses and nuclear pleomorphism, DF has an aggressive nature, presenting as a destructive growth causing entrapment of neuro-vascular bundles, sinusitis, or trismus. Some cases of DF show mutations in the adenomatous polyposis coli pathway shown by nuclear localization of the ß-catenin protein. Few reports showed an association with tuberous sclerosis, though most of these cases were sporadic. We discuss a rare case of desmoplastic fibroma involving the mandible, and a review of the literature of the DF cases involving the gnathic bones.

[Desmoplastic fibroblastoma: a clinicopathologic analysis of 7 cases].

Objective: To investigate the clinical features, immunohistochemical and differential diagnosis of desmoplastic fibroblastoma. Methods: The clinical data and pathology features of 7 cases of desmoplastic fibroblastoma were collected and immunohistochemical study were carried out in all cases with a review of the literatures. Results: There were 2 males and 5 females, with age ranging from 31 to 71 years (average and mean age were 59 and 61 years, respectively). The tumors were located in extremities and abdomen (left toe and right toe, right foot back, left leg and right thigh, right forearm and left hepatic lobe). Clinically, the tumors presented as slow growing painless masses of long standing duration. Grossly, the tumors were well-circumscribed with firm, white to gray cut-off surface. Tumor size ranged from 1.2 to 4.0 cm in maximum diameter (average 3.0 cm). Microscopically, 2 cases were located in dermis, 4 cases were located in subcutaneous and 1 case was located in liver parenchyma. It was composed of spindle-shaped or stellate cells with a fibroblastic or myofibroblastic appearance, and sparsely scattered in densely fibrous or fibromyxoid background. There was small vascular component in tumor background. At high magnification, the tumor cells were medium size with abundant cytoplasm, and the nucleus were small and always with small nucleoli. In some cases, the tumor cells were slightly larger with enlarged nuclei, but without cellular atypical and mitosis. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, desmin, S-100 protein and CD34, but CKpan was negative. α-SMA showed focal positive in one case. Ki-67 index ranged from 1% to 2%. Four cases were followed-up (ranged from 11 to 21 months, average 16.5 months) and the patients had no recurrence after surgery. Conclusions: Desmoplastic firoblastoma is a rare soft benign tumor. The differential diagnosis includes other benign or low-grade fibroblastic/myofibroblastic lesions.

Desmoplastic fibroblastoma: a clinicopathologic analysis of 7 cases / 中华病理学杂志

Objective@#To investigate the clinical features, immunohistochemical and differential diagnosis of desmoplastic fibroblastoma.@*Methods@#The clinical data and pathology features of 7 cases of desmoplastic fibroblastoma were collected and immunohistochemical study were carried out in all cases with a review of the literatures.@*Results@#There were 2 males and 5 females, with age ranging from 31 to 71 years (average and mean age were 59 and 61 years, respectively). The tumors were located in extremities and abdomen (left toe and right toe, right foot back, left leg and right thigh, right forearm and left hepatic lobe). Clinically, the tumors presented as slow growing painless masses of long standing duration. Grossly, the tumors were well-circumscribed with firm, white to gray cut-off surface. Tumor size ranged from 1.2 to 4.0 cm in maximum diameter (average 3.0 cm). Microscopically, 2 cases were located in dermis, 4 cases were located in subcutaneous and 1 case was located in liver parenchyma. It was composed of spindle-shaped or stellate cells with a fibroblastic or myofibroblastic appearance, and sparsely scattered in densely fibrous or fibromyxoid background. There was small vascular component in tumor background. At high magnification, the tumor cells were medium size with abundant cytoplasm, and the nucleus were small and always with small nucleoli. In some cases, the tumor cells were slightly larger with enlarged nuclei, but without cellular atypical and mitosis. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, desmin, S-100 protein and CD34, but CKpan was negative. α-SMA showed focal positive in one case. Ki-67 index ranged from 1% to 2%. Four cases were followed-up (ranged from 11 to 21 months, average 16.5 months) and the patients had no recurrence after surgery.@*Conclusions@#Desmoplastic firoblastoma is a rare soft benign tumor. The differential diagnosis includes other benign or low-grade fibroblastic/myofibroblastic lesions.

The clinicopathologic characteristics of desmoplastic fibroblastoma in neck and nape / 中国耳鼻咽喉头颈外科

OBJECTIVE To describe the clinical, CT or MRI, pathological characteristics of desmoplastic fibroblastoma for helping the diagnosis and treatment. METHODS The clinical data of 4 cases with desmoplastic fibroblastoma were retrospectively analyzed. RESULTS The tumors of all 4 cases were found in the compartment of muscles, and can be removed by conservative excision. The 4 cases were followed up for 38, 7, 6, 3 months respectively, and no local recurrences were found. CONCLUSION Desmoplastic fibroblastoma is a benign fibroblastic neoplasm with distinctive clinical, CT or MRI imaging and pathological characteristics. Optimal management is conservative excision with functional preservation. Needle aspiration cytology and immunohistochemistry are of non-diagnostic value.

Fibrous soft tissue tumor of neck and shoulder girdle: MR and CT characteristics / 中华放射学杂志

Objective To evaluate MR and CT imagings of desmoplastic fibroblastoma(DF) and aggressive fibromatosis(AF) in neck and shoulder girdle, and to emphasis upon the MRI findings with pathological correlation. Methods MR and CT appearances of DFs(4 cases) and AFS(3 cases) confirmed by surgical and pathologic findings were retrospective analysed, which were correlated to the pathologic features. Four cases of DF were male patients, with a median age at 48 years. Two cases of AF were female (20,29 years respectively), and another AF patient was male (79 years). Results (1) Four DFs were round mass located in the intermuscle space of neck, and the margins were mostly well-circumscribed. Envelope was seen in one lesion on MRI. On unenhanced CT, the masses showed homogeneous low attenuation (2 cases) or slightly low attenuation with several heterogeneous areas in it (1 cases). Enhancement was not obvious on post-contrast CT images. MRI studies were done in two patients. Signal intensity (SI) on MR was homogeneous and heterogeneous one lesion respectively on unenhanced T_1-weighted images. Mixed SI as low or high SI nodes within isointense compared with muscle on T_2-weighted images. No evident enhancing was noted after administration of gadopentetate dimeglumine. (2) All three lesions of aggressive fibromatosis arose from musculoaponeurotic structures. The masses were irregular margin and appeared elongated, which oriented in the direction of the muscle bundle. On unenhanced CT examination, the masses had poorly defined and isodense relative to adjacent musculature (2 cases). On T_1-weighted images, three masses consisted of poor margination and isointensity relative to adjacent muscle. On T_2-weighted images, two lesions were heterogeneity of signal, which predominantly slightly high in SI with strip- or node-shape low SI, and one lesion had homogeneous high SI. On post-contrast T_1-weighted images, all lesions showed marked enhancement, heterogeneity of signal and irregular margin appeared distinctly. Of them, two masses had claw-shape invasive margin. Conclusion Both DF and AF, the age range of affected persons different, were fibroblastic origin soft-tissue neoplasms. The distinct imaging characteristics of these two disorders were related to their different [JP3]pathologic features. Concerning the effect of diagnose and differential diagnose, MRI was predominance over CT. [JP]