Fibromatosis subescapular como causa de escápula alada. Presentación de un caso y revisión bibliográfica / Subscapularis fibromatosis as a cause of winged scapula. Case report and literature review

La escápula alada suele producirse por lesiones neurológicas tanto del nervio espinal como del nervio torácico largo. La aparición a causa de un tumor ventral de la escápula dificulta su diagnóstico inicial. Presentamos el caso de una mujer joven, con limitación para la rotación externa del hombro, sin antecedentes traumáticos conocidos, evolución progresiva e imagen compatible con una lesión de partes blandas dependiente de la aponeurosis del músculo subescapular confirmada por biopsia como un tumor desmoide. Aunque se trata de un tumor benigno y autolimitado, tiene una inquietante alta tasa de recidivas después de la resección, por lo que se dispone de numerosos tratamientos y muchos grupos optan por hacer un seguimiento médico estrecho de los factores pronósticos y las limitaciones funcionales del paciente, con lo que obtienen resultados satisfactorios y, en algunas series, superiores a los del tratamiento quirúrgico. Nivel de Evidencia: IV

Winged scapula is usually caused by neurological injuries to both the spinal nerve and the long thoracic nerve. Its presence as a result of a ventral scapular tumor makes initial diagnosis difficult. We present the case of a young woman with limited external shoulder rotation, no known traumatic history, and images consistent with a soft tissue lesion dependent on subscapular muscle aponeurosis, which was confirmed by biopsy as a desmoid tumor. Although it is a benign, self-limiting tumor, it has an alarmingly high rate of relapse after resection, so many treatments are available, and many teams choose to closely monitor the patient's prognostic factors and functional limitations, obtaining satisfactory outcomes and, in some series, superior to those of surgical treatment. Level of Evidence: IV

Tumor desmoide abdominal gigante en puerperio: caso clínico y revisión de la literatura / Giant abdominal desmoid tumor during the puerperium: Case report and review of literature

Introducción. Los tumores desmoides o fibromatosis agresiva corresponden a neoplasias mesenquimales poco frecuentes. Son tumores localmente agresivos que ocurren especialmente en jóvenes, no desarrollan metástasis a distancia, pero se asocian con invasión locorregional y alta tasa de recurrencia después de la resección. Su etiología es desconocida, pero se ha asociado al síndrome de Gardner, trauma, embarazo, estados hiperestrogénicos y puerperio. El objetivo de este artículo fue hacer una revisión sobre el tema a propósito de un caso clínico. Caso clínico. Se presenta el caso de una paciente puérpera con progresivo y rápido aumento del volumen abdominal. Se realizó una tomografía computarizada de abdomen y pelvis que confirmó la presencia de una masa intraperitoneal bien definida. La paciente fue operada con escisión de la masa y confirmación histológica de tumor desmoide a partir de la muestra de patología. Discusión. Los tumores desmoides tienen una incidencia de 2 a 4 casos por millón de habitantes por año, con leve predominio en el sexo femenino y representan menos del 3 % de los tumores de partes blandas. Aunque el tumor se puede ubicar a nivel intraabdominal o en la pared, la ubicación más común es en las extremidades. Conclusiones. La sospecha y detección del tumor desmoide es fundamental, así como su adecuado estudio, para determinar el tratamiento quirúrgico como fue realizado en este caso

Introduction. Desmoid tumors or aggressive fibromatosis correspond to rare mesenchymal neoplasms. They are locally aggressive tumors that occur especially in young people, they do not develop distant metastases, but are associated with locoregional invasion and a high recurrence rate after resection. Its etiology is unknown, but it has been associated with Gardner syndrome, trauma, pregnancy, hyperestrogenic states, and puerperium. The objective of this article was to review the topic based on a clinical case. Clinical case. The case of a puerperal patient with progressive and rapid increase in abdominal volume is presented. An abdominal and pelvic CT scan was performed, which confirmed the presence of a well-defined intraperitoneal mass. The patient underwent surgery with excision of the mass and histological confirmation of a desmoid tumor from the pathology sample. Discussion. Desmoid tumors have an incidence of 2 to 4 cases per million inhabitants per year, with a slight predominance in females, and represent less than 3% of soft tissue tumors. Although the tumor can be located intra-abdominal or in the wall, the most common location is in the extremities. Conclusions. The suspicion and detection of the desmoid tumor is essential, as well as its adequate study to determine the surgical treatment as it was done in this case

Does a Vertebra Fracture Cause a Tumor? A Dorsolumbar Paraspinal Desmoid Tumor: Case Report

Extraabdominal desmoid tumors are uncommon soft-tissue tumors. The etiology of the tumor is still unclear. Injury is one of the etiological factors of soft-tissue tumors. A 41-year-old female patient who had a traumatic vertebral body fracture on the thoracic spine was treated conservatively. Two and a half years later, she presented a painful, palpable swelling on the thoracolumbar region. In the present report, was discuss the patient, who underwent a surgery to remove the desmoid tumor (aggressive fibromatosis), within the context of the current literature. The literature on desmoid tumor caused by a trauma is rare. This is the first case that demonstrates an extraabdominal desmoid tumor following a spinal fracture. The swelling on the region of the trauma must be examined carefully and desmoid tumor must be kept in mind as a possible diagnosis.

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.

Fibroma desmoplástico / Desmoplastic fibroma

El Fibroma Desmoplástico es una patología poco frecuente y de localización mas conocida en maxilar inferior y pocos casos en tibia, fémur y otros huesos. En rótula no se encontraron casos en la abundante bibliografía consultada. Se presenta un caso en un paciente de 15 años que después de un año de tratamiento médico y fisiokinésico se hace RMN y TAC encontrando una imágen osteolítica en rótula izquierda, por lo que después de interconsulta con Ortopedista y Traumatólogo Infantil, se resuelve tratamiento quirúrgico. El objetivo de esta presentación es llamar la atención sobre dolor que se interpreta como tendinitis cuadricipital, con Rx. neg. y dolor en borde proximal de rótula y borde externo de la misma, pero los estudios mencionados nos orientan a una patología muy poco frecuente. Además se destaca que no se encontró en la bibliografía antecedente de otro caso similar en rótula. Se hace resección con margen de seguridad de la zona afectada incluyendo hueso y cartílago articular. La evolución fue satisfactoria, controlado a los 3 años no hay dolor ni limitación funcional. Tipo de estudio: Reporte de caso. Nivel de evidencia: IV

The Desmoplastic Fibroma is an unfrequent pathology and the most common location is in the lower jaw (there are a few cases in the tibia, femur and other bones). There wasn't found a case in the kneecap, despite the extensive bibliography consulted. It is presented a case of a 15 years old patient who after a year of medical and physio-kinesic treatment, underwent MRI and CT scans, finding an osteolytic image in the left patella. After consulting with an Orthopedist and a Child Traumatologist, it was resolved a surgical treatment. The purpose of this presentation is to draw attention to a pain in the proximal and outer edges of the patella interpreted as quadriceps tendonitis, without seeing anything in a radiography. It was made a resection, with a safety margin of the affected area, including bone and articular cartilage. There was a satisfying evolution, checked after 3 years, without pain or functional limitation. Type of Study: case report. Level of evidence: IV

Avaliação das mutações nos códons 41 e 45 do gene codificante da betacatenina em pacientes portadores de tumor desmoide esporádico e sua associação com o prognóstico / Evaluation of mutations in codons 41 and 45 of the Beta-Catenin encoding gene in patients with sporadic desmoid tumors and its association with prognosis

O tumor desmoide (TD) é uma proliferação fibroblástica clonal rara, caracterizada por crescimento infiltrativo e tendência à recidiva local, embora incapaz de metastatizar. A maioria desses tumores é esporádica e contém mutações no gene que codifica a proteína betacatenina. Três mutações em dois códons do gene CTNNB1 foram identificadas como mais frequentes. O tratamento cirúrgico dessa doença tem se mostrado um verdadeiro desafio, e a identificação de fatores prognósticos poderia ajudar na caracterização de grupos com maior ou menor risco para desenvolver recidiva local após esse tratamento. O objetivo deste estudo foi avaliar mutações nos códons 41 e 45 do gene CTNNB1 em pacientes portadores de tumores desmoides esporádicos tratados com cirurgia, bem como avaliar a possível influência de variáveis clínicas, demográficas e moleculares na sobrevida livre de recidiva. Materiais e Métodos: Foi conduzido estudo retrospectivo, unicêntrico, transversal. Foram incluídos pacientes com diagnóstico de tumor desmoide submetidos à ressecção cirúrgica, que tinham dados clínicos e material biológico disponíveis. A correlação entre as variáveis qualitativas foi feita pelo teste qui-quadrado ou teste exato de Fisher; a comparação das variáveis quantitativas pelo teste U de Mann-Whitney ou pelo teste de Kruskal-Wallis; a análise de sobrevida livre de recidiva pela técnica de Kaplan-Meier; e os riscos relativos pela técnica de regressão de Cox uni ou multivariada. Resultados: No período de 1980 a 2015, foram identificados 107 pacientes. A maioria era do sexo feminino (58,9%), de etnia branca (78,9%), tinha idade entre 25 e 50 anos (58,9%), com lesões que se localizavam fora das extremidades (63,4%), apresentando tamanho menor que 10 centímetros (76,4%) e todos foram tratados cirurgicamente. O tempo de seguimento médio foi de 134 meses, a taxa global de sobrevida livre de recidiva (SLR) foi de 74,5% em 2 anos, 65,1% em 5 anos, 63,7% em 10 anos e 59,5% em 20 anos. Dos 107 pacientes que possuíam informações clínicas, foram obtidas 71 amostras tumorais para análise molecular que foi realizada utilizando NGS. Destes, 61 pacientes (85,9%) apresentavam alguma mutação nos códons 41 ou 45 do éxon 3 do gene CTNNB1 e 10 espécimes (14,1%) não tinham qualquer mutação nesses códons. Trinta e três (46,5%) pacientes apresentaram mutação T41A 16 (22,5%) S45F, 6 (8,5%) S45P, 5 (7%) T41A e S45F e 1 (1,4%) T41A e S45F, o valor médio da frequência alélica foi de 16,54%. Os pacientes com mutação S45F apresentaram taxa de sobrevida livre de recidiva em dois, cinco e dez anos menor do que os demais pacientes não mutados ou com outro tipo de mutação (26,7% 13,3% e 13,3% versus 78,4%, 68,1% e 65,4%, respectivamente). Na análise multivariada, tipo de mutação (RR = 5,25 IC [ 2,27­12,15]; p < 0,0001) e topografia (RR = 3,15 IC [1,35­ 7,33]; p = 0,008) foram fatores independentes para o risco de recidiva e o modelo prognóstico proposto evidenciou que os pacientes com alto risco (um ou dois fatores) podem apresentar até 8,36 vezes mais risco de recidiva local após o tratamento cirúrgico que os demais pacientes (IC [2,87­24,6]; p = 0,0001). Os resultados do presente estudo permitem concluir que o sequenciamento de segunda geração é um método adequado para detectar as mutações nos pacientes com tumores desmoides, e a mutação S45F esteve associada com maior risco de recidiva local. Em conjunto com outros fatores clínicos, a presença dessa mutação pode identificar um subgrupo de pacientes com elevado risco de recidiva. Esse achado pode auxiliar na indicação de cirurgias extensas e mutilantes, evitando a morbidade para um grupo específico de pacientes. Todavia, tais achados devem ser validados em uma coorte mais ampla de pacientes

The desmoid tumor (DT) is a rare (mono)clonal fibroblastic proliferation characterized by infiltrative growth with tendency to local recurrence although unable to metastasize. Most of these tumors are sporadic and contain mutations in the gene encoding Beta-Catenin. Three mutations in two codons of the CTNNB1 gene were identified as being more frequent. Surgical treatment of this disease has been revealed to be a real challenge and the identification of prognostic factors would help in the identification of groups with higher or lower risk to develop local recurrence after this treatment. The objective of this study was to evaluate mutations in codons 41 and 45 of the CTNNB1 gene in patients whose tumors were treated with surgery as well as to assess clinical and demographic variables and correlate them with relapse-free survival. Materials and Methods: a retrospective, unicentric, cross-sectional study was conducted. Patients diagnosed with desmoid tumor who were submitted to surgery, had clinical data and biological material available were included. The correlation between the qualitative variables was made by the chi-square test or Fisher exact test; the quantitative variables comparison by the Mann-Whitney U test or Kruskal-Wallis test; the relapse-free survival by Kaplan-Meier; and relative risks by the univariate or multivariate Cox regression technique. Results: 107 patients were identified from 1980 to 2015. The majority were female (58.9%), white (78.9%) 25-50 years old (58.9%), their lesions were located outside the extremities (63.4%), measured less than 10 centimeters (76.4%) and they were all surgically treated. Mean follow up period was 134 months, overall relapse-free survival rate (RFS) was 74.5% in 2 years, 65.1% in 5 years, 63.7% in 10 years and 59.5% in 20 years. From 107 patients who had clinical information, 71 samples were obtained for molecular analysis. Among these, 61 patients (85.9%) had mutations at codons 41 or 45 of exon 3 of the CTNNB1 gene and 10 specimens (14.1%) had no mutations at these codons. Thirty-three (46.5%) patients had mutation T41A 16 (22.5%) S45F, 6 (8,5%) S45P, 5 (7%) T41A and S45F and 1 (1,4%) T41A and S45F, the mean of allele frequency was 16.54%. Patients with mutation S45F had a relapse-free survival rate of two, five and 10 years smaller than the patients with no mutations or with a different mutation (26.7% 13.3% and 13.3% versus 78.4%, 68.1% and 65.4%, respectively). In the multivariate analysis, type of mutation (RR = 5,5 IC[2.27 ­ 12.15]; p < 0.0001) and topography (RR = 3.15 IC [1.35 ­ 7.33]; p = 0.008) correlated with risk of relapse and the proposed prognostic model demonstrated that high risk patients (one or two factors) may present a greater risk (up to 8.36 times) of local recurrence after surgical treatment than other patients (IC[2.87 ­ 24.6]; p = 0.0001). The results of the present study allow us to conclude that second generation sequencing is a suitable method to detect mutations in patients with desmoid tumors and the S45F mutation has been associated with an increased risk of local recurrence. Combined with other clinical factors, the presence of this mutation may identify a subgroup of patients with a high risk of relapse. This finding may support the indication of extensive and mutilating surgeries, avoiding morbidity for a specific group of patients. Nevertheless, such findings should be validated in a broader cohort of patients

Fibroma desmoplásico: tumor benigno, con alto potencial de infiltración local / Desmoplasic fibroma: benign tumor, with high local infiltration capacity

El fibroma desmóplasico es una neoplasia benigna del tejido conjuntivo que tiene alta capacidad de infiltrar los tejidos adyacentes. En este artículo se presenta un caso clínico de una paciente de 3 a±os de edad con un fibroma desmoplßsico en la región sinfisiaria y en el cuerpo mandibular. El tratamiento fue la hemimandibulectomía y la colocación de injerto óseo autólogo y placa de reconstrucción. Se realizó una revisión de la literatura de este tumor benigno poco frecuente de la región maxilo facial...

Desmoplasic fibroma is a benign connective tissue tumor with high local infiltration capacity. This article presents a case of a 3-yearoldgirl who presented a desmoplasic fibroma in the mandible symphysis and body. The treatment was radical resection and placement of an autologous bone graft and reconstruction plate. A review of the published literature was done respect to this rare benign tumor of the maxillofacial region...

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.

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

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 ameloblastoma: a report of two clinical cases

Desmoplastic ameloblastoma is a relatively rare histological variant of ameloblastoma with specific clinical, radiological, and histological features. Although radiographic examination of ameloblastomas usually reveals unilocular or multilocular radiolucency, desmoplastic ameloblastoma may appearas a mixed radiopaque-radiolucent lesion resembling benign fibro-osseous lesions. Histologically, desmoplastic ameloblastoma is characterized by small nests and strands of “compressed”odontogenic epithelium supported by pronounced collagenized stroma. This report describes two cases of desmoplastic ameloblastoma in the anterior maxilla of a female patient and the anterior mandible of a male patient, mimicking clinically as an odontogenic cyst.

Reporte de un caso de tumor desmoplásico de células pequeñas redondas / Case report: patient with round cell desmoplastic tumor

Introducción: El tumor de células pequeñas redondas desmoplásico (TCPRD), es una neoplasia agresiva maligna poco frecuente que afecta a adolescentes y adultos jóvenes. Usualmente son intraabdominales asociados con un mal pronóstico. Existen algunas publicaciones donde incluyen manifestaciones paratesticulares. Caso: Reportamos un caso de un joven de 29 años que consulta por aumento de volumen del hemiescroto izquierdo de 3 meses de evolución. Al examen destacaba 2 nódulos duros uno en relación al polo inferior y el otro al epidídimo de aproximadamente 2 cm. Alfafetoproteína y beta HCG eran normales. Se decide exploración quirúrgica donde biopsia rápida confirma tumor que se origina en las envolturas y que infiltra hacia el testículo. La biopsia definitiva informó tumor desmoplásico de células pequeñas redondas. El estudio de extensión con tomografía axial computarizada y radiografía de tórax no mostró metástasis. El paciente recibió 2 ciclos de QMT con ciclofosfamida, etoposido, adriamicina y cisplatino, con buena tolerancia, evolucionando con depresión medular moderada que se recupera. Actualmente 6 años después del diagnóstico el paciente se encuentra en remisión completa. Discusión: Al parecer y según los últimos reportes de la literatura, la ubicación paratesticular ha mostrado mejor pronóstico en comparación con los tumores abdominales. Se debe incluir este diagnóstico diferencial al enfrentarse con tumores paratesticulares.

Introduction: Small round cell desmoplastic tumor (SRCDT) is an infrequent malignant tumor that affects adolescents and young adults. Usually they occur in the abdomen. Paratesticular manifestations have been reported. Case report: A 29 year old male presented with a 3 month history of a mass in the left scrotum. Physical exam showed 2 hard nodules in the scrotum. Serum levels of alpha-fetoprotein and beta-HCG were normal. The patient was submitted to surgery. Frozen section confirmed a tumor arising in the paratesticular area with involvement of the testis. Permanent sections showed a SRCDT. CT scans and chest x-rays showed no metastases. The patient received two courses of ciclophosphamide, etoposide, adryamicin and cisplatinum. Treatment was well tolerated. The patient is in complete remission at 6 years following the diagnosis. Discussion: Paratesticular location seems to have a better prognosis compared to intraabdominal tumors. SRCDT should be included in the differential diagnosis of paratesticular tumors.

Collagenous fibroma ( desmoplastic fibroblastoma ) of alveolar bone: a case report

O fibroma colagenoso (fibroma desmoplásico) é um tumor de tecido mole raro de origem fibroblástica. Na mucosa oral apenas dois casos foram descritos pela literatura. Nós descrevemos o caso de uma mulher de 42 anos de idade, cuja queixa era uma lesão indolor, de crescimento lento sob sua prótese total superior. Os achados histológicos da lesão incluíram fibroblastos escassos, estrelados ou fusiformes, dispersos em um estroma rico em colágeno. As células lesionais foram positivas para vimentina, mas rara marcação foi observada para actina músculo liso e fator XIIIa. A excisão cirúrgica total foi realizada e a recidiva da lesão não é esperada.

Tumor desmoplasico de celulas pequeñas redondas otra causa de masa abdominal en niños / Desmoplastic tumor of small round cell in child

Se presenta el caso de un niño de 11 años con diagnóstico comprobado de tumor desmoplásico de células pequeñas redondas, manifestado como masa hipogástrica

Fibroma desmoplástico (desmóide) nos ossos / Desmoplastic fibroma (desmoid) in bones

Os autores apresentam 12 casos de fibroma desmóide, 9 intra-ósseos e 3 periostais, dentre cerca de 4.550 casos do Registro de Tumores Ósseos do Departamento de Patologia da Santa Casa de São Paulo. A história clínica variou de 8 meses a 15 anos, com queixa de dor, tumor e progressiva impotência funcional. Os pacientes tinham idade de 12 a 58 anos. A localização do tumor também era variada. As formas intra-ósseas eram no fêmur e um na ulna, no úmero, no osso cubóide do pé, no acetábulo e na décima vértebra torácica. Nos casos de crescimento periostal, um no antebraço, os dois outros na escápula e na asa do ilíaco. Ao exame radiográfico o tumor apresentou densidade de partes moles, alternando áreas de esclerose reacional e de osteólise. O diagnóstico definitivo foi o exame anatomopatológico. O tratamento em todos os casos foi cirúrgico, sempre que possível com ressecção ampla com margem de tecidos normais. Em três a recidiva ocorreu entre dez meses e quatro anos. No caso de localização vertebral, por ter sido realizada ressecção intralesional, a recidiva ocorreu um ano e sete meses depois. Pela raridade de comprometimento, pelo interesse ortopédico dos casos e pela correlação dos exames radiográficos com a anatomia patológica, os autores resolveram divulgar esta casuística.