Ultrasonographic findings of desmoid fibromatosis / 中国医师杂志

Objective:To explore the ultrasonographic features of desmoid fibromatosis and provide evidence for clinical management.Methods:The ultrasonic images and clinical data of 22 cases of desmoid fibromatosis confirmed by pathology in the Union Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology from 2016 to 2021 were retrospectively analyzed. The location, size, shape, boundary, internal echo, posterior acoustic enhancement, relationship with surrounding tissues and blood flow of the lesion were evaluated.Results:The maximum diameter of lesions in 22 patients ranged from 0.8 cm to 11.3 cm (5.2±2.4)cm. (1) There were 17 cases of extra-abdominal shape: 10 cases of irregular shape, 4 cases of spindle shape, and 3 cases of oval shape. In 10 cases, the lesion boundary was not clear, and the lesion was infiltrated along the muscle, fascia or adipose tissue. Uneven hypoechogenicity was found in 11 cases, and strip or patchy strong echo was found in 4 cases. 5 cases with posterior echogenicity enhancement; color Doppler flow imaging (CDFI) blood flow classification: grade 0 in 2 cases, grade 1 in 7 cases, grade 2 in 7 cases, and grade 3 in 1 case. (2) Abdominal wall type in 5 cases: oval shape in 2 cases, spindle shape in 2 cases, irregular shape in 1 case; In 3 cases, the boundary was not clear and was infiltrative along the muscle. Inhomogeneous hypoecho was found in 4 cases. Posterior echogenicity was enhanced in 1 case. CDFI blood flow classification: grade 1 in 2 cases, grade 2 in 2 cases, grade 3 in 1 case.Conclusions:The sonograms of typical desmoid fibromatosis have certain features. Combined with the patient′s history and clinical manifestations, they can provide an important basis for the clinical management of the disease.

Multimodal ultrasonic diagnosis of breast fibromatosis originating from breast parenchyma / 中国医师杂志

Objective:To analyze the imaging characteristics of invasive fibromatosis from breast parenchyma, and to explore the clinical value of multimodal ultrasound.Methods:The two-dimensional, color Doppler and elastic imaging sonographic manifestations and pathological features of 12 cases of breast invasive fibromatosis included in the Affiliated Hospital of Jining Medical College from October 2015 to October 2020 were studied retrospectively.Results:Two dimensional ultrasound showed that 12 cases of breast invasive fibromatosis grew in parallel, with different sizes, unclear boundary and no complete capsule. The edge morphology of 7 cases (7/12) showed crab foot like changes; The tumor showed solid heterogeneous hypoechoic, punctate hyperechoic in 3 cases (3/12), and echo attenuation behind the tumor in 3 cases (3/12); The blood flow in the tumor was mainly grade 1-2 (9/12). Twelve tumors were examined quantitatively by virtual touch tissue quantifification. The shear wave velocity was (3.08±0.75)m/s. The diagnostic accuracy of multimodal ultrasound in invasive fibromatosis of breast (10/12) was significantly higher than that of conventional ultrasound (3/12, χ 2=8.224, P=0.004). The gross manifestation of the tumor: the section was gray or gray yellow, with unclear boundary and no capsule; Microscopic findings: fascicular fibroblasts and myofibroblasts proliferated, accompanied by varying degrees of glassy degeneration, acellular atypia, nuclear division and necrosis. Conclusions:Conventional ultrasound is difficult to distinguish invasive fibromatosis and malignant tumor of breast, and its ultrasonic manifestations are closely related to pathological features. The combined application of multimodal ultrasound can significantly improve the diagnostic coincidence rate of the disease and has high clinical application value.

Mesenteric fibromatosis: report of 7 cases / 中华普通外科杂志

Objective To invastigate the etiology,diagnosis and treatment of mesenteric fibromatosis.Methods 7 cases of mesenteric fibromatosis was admitted into our hospital from Jan 2012 to Jan 2018.Reslut Among the 7 patients,there were 2 males and 5 females,with an average age of (40 + 16)years,4 cases had had a history of abdominal surgery and 1 case was of familial polyp,the symptoms of organ compression were the first manifestation in 4 cases,abdominal mass as the first manifestation in 2 cases.The lesions were located on small mesentery in 5 cases,on mesenteric membrane in one,and on mesorectal membrane in one.All 7 patients underwent extensive tumor resection and the margins were negative,one of the tumors was located at the level of the duodenum,invading the superior mesenteric vein and the transverse mesocolon.Immunohistochemistry results:[3-catenin (+)、Ki-67 (+),CD117 (-),CD34(-)in7 cases,SMA(+)in5 cases,Desmin (+) in4 cases,ER (+) in3 cases.Conclusion Most patients of mesenteric fibroma have a history of previous abdominal surgery,seconded by organ compression symptoms.β-catenin (+),Ki-67 (+) and CD117 (-),CD34 (-) contribute to pathological diagnosis,Surgery-based comprehensive treatment can achieve therapeutic effect.

Extra?abdominal desmoid?type fibromatosis: MR findings and related features of recurrence / 中华放射学杂志

Objective To explore the MR findings of extra?abdominal desmoid?type fibromatosis and the related features of recurrence. Methods MRI and clinical data of 36 cases of extra?abdominal desmoid?type fibromatosis proved by surgical pathology were retrospectively reviewed between March 2010 and October 2016.The ages of the cases ranged from 10 years to 68 years. 14 males and 22 females were included with sex ratio 1∶1.57.the courses of the disease were varied from 1 day to 8 years. The stage was based on Church′s method. 3 cases were only with plain scan and other 33 cases with contrast enhancement. Sagittal or coronal T1WI, STIR?T2WI, PdWI and contrast enhanced T1WI in horizontal, sagittal and coronal view were examined on MR siganl 1.5T or 3.0T of GE Company. Two groups were divided based on the recurrence or not after surgery. The clinical and MR features were analyzed between the two groups with χ2 test, t test or Spearman rank correlation analysis according to the data characteristics. Results Compared with signal intensity of muscle, 34 cases were isointense and 2 cases isointense with hypointense area on T1WI; 34 cases were hyperintense and 2 cases hyperintense with hypointense area on T2WI. On contrast enhanced imaging, 22 cases were markedly enhanced, 7 cases moderately enhanced and 4 cases mildly enhanced.15 cases was with recurrence and 21 cases without recurrence. Infiltration of surrounding tissue was shown in 14 cases(38.9%), 13 cases with recurrence and 1 cases without recurrence. Band sign was shown in all cases. Tentacle sign was shown in 18 cases (50.0%), 15 cases with recurrence and 3 cases without recurrence. Split fat sign was shown in 12 cases (33.3%) and all without recurrence. Fascial tail sign was shown in 15 cases (41.7%), 13 cases with recurrence and 2 cases without recurrence. Hypointense ring around the tumor was shown in 21 cases (58.3%), 2 cases with recurrence and 19 cases without recurrence. The P value of tentacle sign, split fat sign, hypointense ring around the tumor, fascial tail sign and infiltration of surrounding tissue was less than 0.05. Gender, location and the degree of contrast enhancement was without difference of statistical significance between the two groups. There was significant correlation of the recurrence rate with the courses and the stage of the disease(χ2=7.264 , r=0.994 , P<0.05). Conclusions Tentacle sign, fascial tail sign and infiltration of surrounding tissue were attributed to the estimate of recurrence of extra?abdominal desmoid?type fibromatosis, and Be features contributes to the diagnosis and therapy of extra?abdominal desmoid?type fibromatosis.

Large desmoid tumors in familial adenomatous polyposis: a successful outcome

Desmoid tumors develop from connective tissue, fasciae, and aponeuroses, and may occur in the context of familial adenomatous polyposis or may arise sporadically; also, they may be extra-abdominal, intra-abdominal, or located in the abdominal wall. These benign tumors have a great aggressiveness with a high rate of local recurrence. Familial adenomatous polyposis is an inherited condition with autosomal dominant transmission, and is characterized by the development of multiple colonic and rectal adenomatous polyps, as well as desmoid tumors. We present the case of a 54-year-old woman with germline APC gene mutation, who underwent a total colectomy, subsequently developing two large infiltrative solid intra-abdominal lesions consistent with desmoid tumors. Medical treatment with Cox-2 inhibitors was initiated without result. She was submitted to resection for intestinal obstruction, but developed local recurrence. The lesions were also unresponsive to tamoxifen, and chemotherapy was initiated with dacarbazine plus doxorubicin, switching to vinorelbine plus methotrexate, achieving a good response in all lesions after 12 months. The approach to these intra-abdominal lesions should be progressive, beginning with observation, then a medical approach with non-steroidal anti-inflammatory drugs or with an anti-hormonal agent. Afterwards, if progression is still evident, chemotherapy should be started. Surgery should be reserved for resistance to medical treatment, in palliative situations, or for extra-abdominal or abdominal wall desmoids tumors.

Extra-abdominal desmoid tumor: analysis of 23 consecutive cases in a single institution / Tumor desmoide extra-abdominal: análise de 23 casos consecutivos em uma única instituição

ABSTRACT Objective: Extra-abdominal desmoid tumor (EDT) is a rare condition, caused by proliferation of fibroblasts. Despite being a benign tumor, it is locally aggressive and has unpredictable clinical behavior. The objective of this study is to present the clinical outcomes of patients with EDT treated surgically between 1995 and 2016. Methods: This is a retrospective series of 23 patients with histopathological diagnosis of EDT that underwent surgery at the orthopedic oncology service of our hospital. The information was obtained from the institute's clinical and pathology reports. Results: A total of 223 medical records with histopathological reports were evaluated. Only 23 cases of EDT were included in the present study. The mean age was 22.5 years. Twelve (52.2%) patients had the tumor on the lower limbs, seven (30.4%) on the upper limbs and four (17.4%) cases were reported on the back. Five (21.7%) patients had tumors measuring less than 5 cm, while eighteen (78.3%) patients had tumors measuring more than 5 cm. All patients underwent surgery as the definitive treatment in our institute. Twelve (52.2%) cases presented negative margins (NM) and eleven (47.8%) cases had positive margins (PM). Local recurrence (LR) occurred in eleven (47.8%) patients. Conclusion: Impairment of the surgical margin was the only prognostic factor found for LR of EDT. Level of Evidence IV, Case Series.

RESUMO Objetivo: O tumor desmoide extra-abdominal (TDE) é raro, formado por proliferação de fibroblastos. Apesar de ser um tumor benigno, é localmente agressivo e tem comportamento clínico imprevisível. O objetivo desta pesquisa é apresentar os resultados clínicos obtidos nos pacientes com TDE, tratados cirurgicamente entre 1995 e 2016. Métodos: Trata-se de uma série retrospectiva de 23 pacientes com diagnóstico anatomopatológico de TDE, tratados cirurgicamente no serviço de oncologia ortopédica de nosso hospital. A informação foi obtida dos relatos clínicos e patológicos do instituto. Resultados: Um total de 223 prontuários com relato anatomopatológico foi revisado. Apenas 23 casos de TDE foram incluídos no presente estudo. A média de idade foi de 22,5 anos. Doze (52,2%) casos se localizaram nos membros inferiores, sete (30,4%) casos nos membros superiores e quatro (17,4%) casos se localizaram no dorso. Cinco (21,7%) casos tinham tamanho < 5 cm e 18 (78,3%) casos tinham tamanho > 5 cm. Todos os pacientes receberam tratamento cirúrgico como terapia definitiva no instituto. Doze (52,2%) casos apresentaram margens livres (ML) e onze (47,8%) casos tinham margens comprometidas (MC). A recorrência local (RL) ocorreu em onze (47,8%) pacientes. Conclusão: O comprometimento da margem cirúrgica foi o único fator de prognóstico encontrado para a RL do TDE. Nível de Evidência IV, Série de Casos.

Tumor desmoide / Desmoid tumor

Introducción. El tumor desmoide grado I, es una patología rara, se consideraba la intervención quirúrgica como primera opción, pero su recidiva posquirúrgica tendía a obscurecer la evolución satisfactoria ya que este tipo de tumores tienden a comprometer planos cada vez más profundos, en algunos casos ha provocado la amputación de extremidades sin lograr éxito, generando un grave impacto psicológico en el paciente y su pronóstico. Caso clínico. Paciente masculino, a los 18 años de edad, presentó una masa en el tercio distal del brazo izquierdo, en Italia le realizan exéresis quirúrgica, la misma que reporta positivo para tumor desmoide, presentando varias recidivas años después, estudios histopatológicos reportan recidiva de tumor desmoide. Actualmente se encuentra en seguimiento con controles tomográficos. Discusión. El tumor desmoide carece de potencial metastásico, con un comportamiento local muy agresivo, las técnicas de imagen permiten realizar su diagnóstico diferencial de otros tumores que afectan los tejidos blandos, su diagnóstico definitivo es con biopsia y su estudio histopatológico. Nuestro paciente a los 18 años se le diagnostica de un tumor desmoide a nivel del brazo izquierdo, presentando por varias ocasiones recidivas posterior a varias exéresis de los mismos, cabe recalcar, que este tipo de tumor tiene una elevada tasa de recurrencia incluso después de una resección completa del tumor; por lo que la cirugía no está indicada como tratamiento de primera elección, indica el manejo expectante está indicado con la estrategia O-E (Observar-Esperar), cuando el paciente es referido al HECAM se decide mantener controles expectantes. Como lo recomienda las ultimas guías de tratmiento.

Introduction. Grade I desmoid tumor is an uncommon pathology where treatment was the surgery as first option, but its post-surgical recurrence tends to obscure satisfactory evolution due to these kinds of tumors have to compromise deep layers, and there are cases where patients needed amputation of their arms or legs without any success; therefore, it causes a huge psychological impact in the patients and their prognosis. Clinical case. An eighteen-year old male presented with a mass in the distal portion of the left arm. In Italy, he had a surgical resection, and the sample was positive for a desmoid tumor, presenting several recurrences years later. Histological studies reports recurrence of desmoid tumor. Is currently in follow-up with tomographic controls. Discussion. The desmoid tumor lacks metastatic potential, with a very aggressive local behavior, the imaging techniques allow its differential diagnosis of other tumors that affect the soft tissues, and its definitive diagnosis is with biopsy and its histopathological study. In summary, our eighteen-year old patient's diagnose was a desmoid tumor on his left arm, presenting several recurrences after several exeresis of the same, it should be emphasized that this type of tumor has a high rate of recurrence even after a complete tumor resection. Thus, surgery is not indicated as first-line of treatment, so it´s recommended expectant treatment based on the O-E strategy (Observe-Wait). When the patient was referred to Hospital de Especialidades Carlos Andrade Marin, the doctors decided to continue with observational management. As recommended by the latest treatment guidelines.

Excision of giant desmoid in the abdominal wall, method of abdominal wall reconstruction, and follow-up of long-termed effect / 中华外科杂志

Objective@#To explore the ideal procedure of excision and repair for giant desmoid in the abdominal wall and long-termed follow-up results.@*Methods@#Clinical and follow-up data of 24 patients with giant desmoid in the abdominal wall underwent radical removal and immediate abdominal wall reconstruction in Diagnostic and Therapeutic Center of Hernia and Abdominal Wall Diseases, First Affiliated Hospital of People′s Liberation Army General Hospital from October 2006 to October 2016 were analyzed retrospectively. Twenty-one female patients with the mean age of 34.6 years and 3 male patients with the mean age of 42.6 years were recruited. The minimal diameter of these tumors was 15 cm, and the maximal diameter was from xiphoid bone to pubic symphysis.@*Results@#All of desmoids were removed radically and proved by the rapid pathologic examination. The size of abdominal wall defect after desmoids removal were 483 (21 cm×23 cm) to 2 100 cm2 (35 cm×60 cm), averaged 945 cm2 (27 cm×35 cm). All of defects were repaired with compound synthetic prosthesis using bridging procedure. Twenty-one patients were recovered smoothly and got primary wound healing. Three patients had prosthesis infected during 1 month postoperatively and 1 patient recovered with conservative therapy, the other 2 patients underwent infected prosthesis removal at 2 weeks and 3 months postoperatively, respectively. Twenty-two patients were followed up with the period of 12 to 121 months and the median period was 63 months. No marginal neoplasm recurrence, incisional hernia, and abdominal wall bulge happened. Eight patients developped fresh desmoids in the abdominal cavity or in the back. Two patients died because of intestinal obstruction due to desmoid infiltration, and the other 6 patients still survived along with stable desmoids.@*Conclusions@#Radical removal for patients with giant desmoid in the abdominal wall is an ideal therapeutic method, and compound synthetic patch can be used to repair huge abdominal wall defect, even the defect compromised all of abdominal wall. The long-termed follow-up results showed these procedures had not put bad influence on the quality of patients′ life.

Fibromatose agressiva (tumor desmoide) associada ao implante mamário: revisão da literatura e apresentação de três novos casos / Aggressive fibromatosis (desmoid tumor) associated with breast implant: literature review and presentation of three new cases

INTRODUÇÃO: As fibromatoses consistem na proliferação de tecido fibroso, na forma de faixas ou nódulos, associadas às fáscias e aponeuroses. Sua variedade agressiva, denominada previamente tumor desmoide, se comporta de forma semelhante a uma neoplasia maligna, com destruição local de tecidos, sem, entretanto, originar metástases. MÉTODOS: Fez-se revisão da literatura de1979 a janeiro de 2017. Dados de três pacientes, operados entre maio de 2010 e agosto de 2015, foram revistos, com proservação até março de 2017. Observaram-se idade, características do implante, via de introdução do mesmo, tempo decorrido entre o implante e o aparecimento da fibromatose, tratamento cirúrgico instituído, acompanhamento clínico e resultados. RESULTADOS: Foram encontrados 24 trabalhos na literatura disponível, versando sobre fibromatose agressiva da parede torácica associada ao implante mamário de silicone, nos quais foram relatados 34 casos. São apresentados três casos de fibromatose agressiva associados ao implante mamário de silicone. Os casos foram tratados com sucesso por ressecção alargada da parede torácica, incluindo pele, musculatura, costelas, fáscia endotorácica e pleura parietal. A reconstrução foi bem-sucedida, realizada com tela aloplástica (Prolene & reg;) recoberta por retalho muscular em dois casos e retalho cutâneo local em um caso. CONCLUSÃO: A associação de fibromatose agressiva e implante mamário é rara. O tratamento deve consistir em cirurgia alargada, removendo-se o implante e toda a área da cápsula adjacente, em conjunto com parte da mama, costelas subjacentes, musculatura intercostal, fáscia endotorácica e pleura parietal. A reconstrução deve ser feita com tela aloplástica associada a retalho muscular ou retalho tegumentar local.

INTRODUCTION: Fibromatoses consists of the proliferation of fibrous tissue, in the form of bands or nodules, associated with fasciae and aponeuroses. The aggressive variety, previously denominated desmoid tumor, behaves similarly to malignant neoplasm, with local destruction of tissues, without, however, producing metastases. METHODS: A literature review was carried out from 1979 to January 2017. Data from three patients, operated between May 2010 and August 2015, were reviewed. Age, implant characteristics, route of introduction, time elapsed between implantation and fibromatosis, surgical treatment, clinical follow-up and results were observed. RESULTS: Twenty-four papers were found in the literature reporting aggressive fibromatosis of the chest wall associated with silicone breast implant. In these studies, 34 cases were reported. Three new cases of aggressive fibromatosis associated with silicone breast implant are now presented. These cases were successfully treated by extensive resection of the chest wall, including skin, musculature, ribs, endothoracic fascia, and parietal pleura. The reconstruction was successful, performed with alloplastic mesh (Prolene & reg;) covered by muscular flap in two cases and local skin flap in one case. CONCLUSION: The association of aggressive fibromatosis and breast implant is rare. The treatment should consist of extensive surgery, removing the breast implant and the entire area of the capsule around it, part of breast, together with the underlying ribs, intercostal muscles, endothoracic fascia and parietal pleura. The reconstruction should be made with an alloplastic mesh, covered by muscular flaps or local skin flap.

Genetic profile, risk factors and therapeutic approach of desmoid tumors in familial adenomatous polyposis / Perfil genético, fatores de risco e abordagem terapêutica dos tumores desmóides na Polipose Adenomatosa Familiar

ABSTRACT Introduction: Desmoid tumors are the main extraintestinal manifestation of FAP, presenting high morbidity and mortality. It is a neoplasia without metastasis capacity, but with infiltrative growth and with a high rate of recurrence. In familial forms, these tumors are associated with a germinal mutation in the APC gene, with a genotype-phenotype correlation influenced by other risk factors. Materials and methods: A review of articles published since the year 2000 in Portuguese, English or Spanish on desmoid tumors in patients with FAP was carried out. A total of 49 publications were included. Results: The site of the mutation in the APC gene is related to the severity of FAP and to the frequency of desmoid tumor. Mutations located distally to codon 1309 are associated with a more attenuated polyposis, but with higher frequency of desmoid tumors. Clinically, these tumors may or may not be symptomatic, depending on their size and location. In their treatment, priority should be given to medical therapy, especially in intra-abdominal tumors, with surgery being the last option if there are no other complications. Discussion: These tumors are associated with certain risk factors: genetic (mutation site), hormonal (estrogenic environment) and physical (surgical trauma) ones. In young women, a later prophylactic colectomy is suggested. Moreover, the laparoscopic approach to prophylactic surgery seems to be an option that reduces surgical trauma and consequently the appearance of desmoid tumors. Conclusion: The step-up medical approach has been shown to be valid in the treatment of intra-abdominal desmoid tumors, and medical treatment should be the first therapeutic option.

RESUMO Introdução: Os tumores desmóides são a principal manifestação extraintestinal da PAF, apresentando elevada morbimortalidade. É uma neoplasia sem capacidade de metastização, mas com crescimento infiltrativo e com alta taxa de recorrência. Nas formas familiares associa-se a uma mutação germinativa no gene APC, havendo uma correlação genótipo-fenótipo influenciada por outros fatores de risco. Materiais e métodos: Foi efetuada uma revisão de artigos publicados desde o ano 2000, em português, inglês ou espanhol, acerca de tumores desmóides em doentes com PAF. Foram incluídas, no total, 49 publicações. Resultados: O local da mutação no gene APC relaciona-se com a gravidade da PAF e frequência de tumor desmóide. Mutações localizadas distalmente ao codão 1309 associam-se a uma polipose mais atenuada, mas a maior frequência de tumor desmóide. Clinicamente podem ser, ou não, sintomáticos, dependendo do seu tamanho e localização. No seu tratamento deve ser dada prioridade à terapêutica médica, sobretudo nos tumores intra-abdominais, colocando a cirurgia como última opção, caso não hajam outras complicações. Discussão: Estes tumores associam-se a determinados fatores de risco: genéticos (local da mutação), hormonais (ambiente estrogénico) e físicos (trauma cirúrgico). Nas mulheres jovens sugere-se a realização de colectomia profilática mais tardiamente. Além disso, a abordagem laparoscópica para a cirurgia profilática parece ser uma opção que diminui o trauma cirúrgico e consequentemente o aparecimento de tumores desmóides. Conclusão: A abordagem médica em step-up mostrou ser válida no tratamento de tumores desmóides intra-abdominais, devendo o tratamento médico ser a primeira opção terapêutica.

Ultrasound performances and misdiagnostic analysis of aggressive fibromatosis / 中国介入影像与治疗学

Objective To explore ultrasound performances and misdiagnostic causes of aggressive fibromatosis (AF).Methods Ultrasound performance and misdiagnostic causes of 45 patients (47 tumors) with AF confirmed by histopathology were analyzed retrospectively.Results Of 45 patients,ultrasound showed lesions of varying size,and the maximum diameter of 36 masses (36/47,76.60％) were more than 3 cm;37 masses (37/47,78.72％) were irregular pale leaf;45 masses (45/47,95.74％) had no complete capsule;45 masses (45/47,95.74％) were shown as interogeneous internal echo mixed with hyperechoic area in the hypoechoic internal;29 cases were diagnosed correctly,16 cases were misdiagnosed,the diagnostic accordance rate was 64.44％ (29/46).Conclusion AF has certain ultrasonic characteristics.Combined with the patient's medical history and physical sign,ultrasound can significantly improve the preoperative detection rate and diagnostic accuracy of AF.

Extra-abdominal desmoid tumor: local recurrence and treatment options

OBJECTIVE: To evaluate the rate of local recurrence of extra-abdominal desmoid tumor and compare the outcomes of surgical treatment and conservative treatment. METHODS: Twenty one patients (14 women and seven men), mean age 33.0±8.7 years old, with a diagnosis of desmoid tumor were evaluated. The mean follow-up period was 58.5±29.0 months. Fourteen cases involved the lower limbs, four cases involved the upper limbs, and three cases involved the trunk. The average tumor size was 12.7±7.5 cm. Of the 21 patients, 14 did not undergo previous treatment and seven patients relapsed before the initial evaluation. Surgical treatment was performed in 16 patients and conservative treatment was performed in five patients. RESULTS: Recurrence occurred in seven patients (33%) and six of them relapsed within the first 18 months. No significant difference was observed between conservative and surgical treatment. However, a significant difference was observed among patients undergoing wide resection and who experienced improved local control. CONCLUSION: The recurrence rate of desmoid tumor was 33.3%. There was no difference in recurrence between conservative and surgical treatment. In surgical treatment, wide margins showed better results for recurrence control. Level of Evidence III. Retrospective Observational Study

Intrathoracic Desmoid Tumor Presenting as Multiple Lung Nodules 13 Years after Previous Resection of Abdominal Wall Desmoid Tumor / 결핵및호흡기질환

Desmoid tumors are rare soft tissue tumors considered to have locally infiltrative features without distant metastasis until now. Although they are most commonly intraabdominal, very few cases have extra-abdominal locations. The origin of intrathoracic desmoid tumors is predominantly the chest wall with occasional involvement of pleura. True intrathoracic primary desmoid tumors with no involvement of the chest wall or pleura are extremely rare. We recently experienced a case of true intrathoracic desmoid tumor presenting as multiple lung nodules at 13 years after resection of a previous intraabdominal desmoid tumor.

Reinserção psicossocial de paciente jovem através de desarticulação interescápulo torácica e hemitoracectomia / Psychosocial rehabilitation of a young patient through interscapular thoracic disarticulation and hemithoracectomy

Na era das cirurgias minimamente invasivas, parece controverso advogar grandes operações. Entretanto, ainda há espaço para grandes intervenções oncológicas. É relatado caso de paciente de 23 anos de idade portador de tumor desmoide em hemitórax direito, com deformidade e erosão de arcos costais, atelectasia parcial do pulmão ipsilateral, compressão e desvio contralateral do mediastino, invasão de nervos do plexo braquial, vasos subclávios e pleura apical direita. Realizada toracectomia, ressecção de tumor em mediastino, amputação do membro superior direito, escapulectomia e reconstrução da parede torácica com tela de márlex e metilmetacrilato e confecção de retalho fasciocutâneo. O paciente encontra-se em seguimento ambulatorial com suas funções diárias quase recuperadas, limitado apenas nas atividades que envolviam o membro desarticulado. Em casos selecionados devem ser consideras grandes intervenções com o objetivo de ressecção R0 (ressecção com margens cirúrgicas livres) e melhora na qualidade de vida, atentando-se sempre para a ressocialização.

In the era of minimally invasive surgery, advocating large operations seems controversial. However, there is still room for major oncologic interventions. This is report of a 23 yearold patient with desmoid tumor in the right hemithorax, deformed and eroded ribs, ipsilateral lung partial atelectasis, contralateral mediastinal compression and shift, invasion ofnerves in the brachial plexus, subclavian vessels and right apical pleura. Thoracectomy was performed with resection of the tumor in the mediastinum, right upper limb amputation, scapulectomy, and chest wall reconstruction with methylmethacrylate and marlex screen, and use of a fasciocutaneous flap. The subject is in follow up as an outpatient andhas almost recovered his daily functions, which are now limited only for activities involving the inarticulate member. Selected cases should be considered for major interventions with aiming at R0 resection (resection with free surgical margins) and improved quality of life, always bearing in mind the need for rehabilitation and socialization.

Mesenteric desmoid-type fibromatosis causing secondary hypertension in a young woman

This report describes an 18-year-old woman presenting with abdominal distension, left flank pain, and hypertension. She had a huge abdominal mass, diagnosed as a mesenteric desmoid-type fibromatosis, causing compression of the left external iliac vessels and ureter, as well as elevated renin concentration and hypertension. After surgical removal of the mass, all signs improved including hypertension.

Intracranial Fibromatosis: A Case Report

Fibromatosis can occur at various sites, but intracranial fibromatosis is exceptionally rare. Here, we report a case of intracranial fibromatosis arising in the suprasellar area of a 52-year-old woman who had undergone a surgery at that site. A computed tomography scan revealed a heavily calcified, highly enhancing, poorly demarcated mass in the left sellar area that extended into the left suprasellar, parasellar areas, and orbital apex and completely encased the left distal inferior cerebral artery. Histologic and immunohistochemical features were compatible with those of fibromatosis, although the cellularity was focally higher than usual. The etiology of extra-abdominal fibromatosis is unknown, but physical injuries such as trauma and irradiation have been reported to be associated with its occurrence. Although fibromatosis is rare in the intracranial area, it should be considered as a differential diagnosis when an intracranial mass occurs at a previously injured site.

Intracranial Fibromatosis: A Case Report

Fibromatosis can occur at various sites, but intracranial fibromatosis is exceptionally rare. Here, we report a case of intracranial fibromatosis arising in the suprasellar area of a 52-year-old woman who had undergone a surgery at that site. A computed tomography scan revealed a heavily calcified, highly enhancing, poorly demarcated mass in the left sellar area that extended into the left suprasellar, parasellar areas, and orbital apex and completely encased the left distal inferior cerebral artery. Histologic and immunohistochemical features were compatible with those of fibromatosis, although the cellularity was focally higher than usual. The etiology of extra-abdominal fibromatosis is unknown, but physical injuries such as trauma and irradiation have been reported to be associated with its occurrence. Although fibromatosis is rare in the intracranial area, it should be considered as a differential diagnosis when an intracranial mass occurs at a previously injured site.

Tumor desmoide abdômino-pélvico / Abdominopelvic desmoid tumor

O tumor desmoide é uma neoplasia não capsulada, localmente agressiva, originária dos fibroblastos dos tecidos músculo-aponeuróticos. Embora ele não tenha comportamento maligno, tal como capacidade de gerar metástases ou de invasão, o tumor desmoide tem uma alta capacidade de crescimento local, causando deformidades nos órgãos adjacentes, dor e, eventualmente, disfunção orgânica, dependendo da área envolvida. Relatamos o caso de um tumor desmoide intra-abdominal de grandes proporções, invadindo órgãos pélvicos em uma paciente de 53 anos. A neoplasia foi totalmente extirpada e, atualmente, cinco anos após a cirurgia, a paciente encontra-se sem sinal clínico ou radiológico de recidiva da lesão.

Desmoid tumor is a noncapsulated neoplasia, locally aggressive, originated from the fibroblasts of the musculo-aponeurotic tissues. Even though with no malignant behavior, such as the ability of generating metastasis or of invasion, the desmoid tumor has a high tendency for local growth, causing deformities in the adjacent organs, pain and sometimes organ dysfunction, depending on the area involved. We report the case of a large intra-abdominal desmoid tumor, invading pelvic organs in a 53 year-old patient. The neoplasia has been totally extirpated and, at the moment, five years after the surgery, the patient presents no clinical or radiologic sign of lesion relapse.

CT and MRI manifestations of aggressive fibromatosis / 中华放射学杂志

Objective To discuss CT and MRI features of aggressive fibromatosis (AF), especially the characteristic signs of MRI, and to evaluate the value of MRI in diagnosing the disease. Methods The CT and MRI studies in 9 cases with pathologically proven AF were retrospectively analyzed. Results Among 9 cases with AF, one cases could not be discovered by CT and all cases could be discovered by MRI. Appearances of lesion were infiltrating in 7 cases and mass-like in 2 cases. All cases were without calcification and lipo-tissue in the lesion and without edema on adjacent muscle structures. A homogeneous density and high density was seen on CT plain scans and high density on contrast enhanced scans. On MRI scans, variable signal intensity was demonstrated on T 1WI, and high signal intensity on T 2WI, as well as increased signal intensity after contrast enhancement. The signal characteristics primarily reflected the underlying histologic composition of the lesions. Conclusion MRI is more excellent than CT in delineating the site, shape, and extent of these lesions. MRI is valuable in detecting and differentiating AF.