RESUMO
Low-grade myofibroblastic sarcoma (LGMS) represents an atypical tumor composed of myofibroblasts with a variety of histological patterns and with a high tendency to local recurrence and a low probability of distant metastases. LGMS has predilection for the head and neck regions, especially the oral cavity. This study aimed to report 13 new cases of LGMS arising in the oral and maxillofacial region. This study included LGMS cases from five oral and maxillofacial pathology laboratories in four different countries (Brazil, Peru, Guatemala, and South Africa). Their clinical, radiographic, histopathological, and immunohistochemical findings were evaluated. In this current international case series, most patients were females with a mean age of 38.7 years, and commonly presenting a nodular lesion in maxilla. Microscopically, all cases showed a neoplasm formed by oval to spindle cells in a fibrous stroma with myxoid and dense areas, some atypical mitoses, and prominent nucleoli. The immunohistochemical panel showed positivity for smooth muscle actin (12 of 13 cases), HHF35 (2 of 4 cases), ß-catenin (3 of 5 cases), desmin (3 of 11 cases), and Ki-67 (range from 5 to 50%). H-caldesmon was negative for all cases. The diagnosis of LGMS was confirmed in all cases. LGMS shows predominance in young adults, with a slight predilection for the female sex, and maxillary region. LGMS should be a differential diagnosis of myofibroblastic lesions that show a proliferation of spindle cells in a fibrous stroma with myxoid and dense areas and some atypical mitoses, supporting the diagnosis with a complementary immunohistochemical study. Complete surgical excision with clear margins is the treatment of choice. However, long-term follow-up information is required before definitive conclusions can be drawn regarding the incidence of recurrence and the possibility of metastasis.
Assuntos
Fibrossarcoma , Humanos , Feminino , Adulto , Masculino , Fibrossarcoma/patologia , Miofibroblastos/patologia , Cabeça/patologia , Diagnóstico Diferencial , BrasilRESUMO
BACKGROUND: Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6-NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. OBJECTIVE: To report our experience with the use of larotrectinib in pediatric patients. METHODS: Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. RESULTS: Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. CONCLUSION: Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations.
Assuntos
Fibrossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Lactente , Humanos , Criança , Recém-Nascido , Fibrossarcoma/tratamento farmacológico , Fibrossarcoma/genética , Fibrossarcoma/patologia , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia , Inibidores de Proteínas Quinases/uso terapêuticoRESUMO
Low-grade fibromyxoid sarcoma (LGFMS) is a malignant neoplasm of soft tissue, which occurs mainly in extremities. In this study, we described a rare case of LGFMS arising in the labial mucosa, in a 7-year-old male patient, who presented a painless multilobulated growth with an evolution time of 12 months. An incisional biopsy was performed and microscopic examination revealed a proliferation of bland regular spindle cells in an alternating myxoid and hyalinized stroma. The tumor cells showed a strong cytoplasmic immunoreactivity for MUC4 and low cell proliferation index measured by Ki-67 antibody. The diagnosis of LGFMS was established and the patient was referred for surgical treatment at a pediatric oncology hospital.
Assuntos
Fibrossarcoma , Criança , Humanos , Masculino , Fibrossarcoma/diagnóstico , Fibrossarcoma/cirurgia , Fibrossarcoma/patologia , Oncologia , Mucosa/patologia , Sarcoma/patologiaRESUMO
PURPOSE: Low-grade myofibroblastic sarcoma (LGMS) is a rare entity with a predilection for the head and neck. There are still no optimal treatment strategies for patients with LGMS. We retrospectively investigated the efficacies of chemotherapy and radiation treatment for patients with LGMS. METHODS/PATIENTS: We obtained data from the Surveillance, Epidemiology, and End Result (SEER) database for 96 patients diagnosed with LGMS between 2001 and 2015. We used Kaplan-Meier curves and log-rank tests to estimate overall survival (OS) and Cox proportional hazard regression to identify prognostic factors. RESULTS: The median age of the patients was 55.0 years. Twenty-two of the patients had LGMS in the head and neck region. Of the 96 patients, 86 (89.6%) received surgical treatment, 28 (29.2%) received radiation treatment, and 20 (10.4%) received chemotherapy. The mean OS was 125.2 [95% confidence interval (CI) 106.3-144.2] months. The 1, 3, 5, and 10-year OS rates were 88%, 77%, 70%, and 59%, respectively. Age greater than 60 years, positive nodal status, and no surgical treatment were independent prognostic factors for patients with LGMS, whereas chemotherapy and radiation treatment were not. CONCLUSIONS: Surgical resection is the most effective therapy for LGMS. Chemotherapy and radiation had limited effects on survival improvement for patients with LGMS. Therefore, chemotherapy and/or radiation therapy should not be routinely performed in LGMS, especially for those with negative margins after surgery.
Assuntos
Fibrossarcoma/tratamento farmacológico , Fibrossarcoma/radioterapia , Doenças Raras/tratamento farmacológico , Doenças Raras/radioterapia , Adulto , Fatores Etários , Idoso , Intervalos de Confiança , Feminino , Fibrossarcoma/patologia , Fibrossarcoma/cirurgia , Neoplasias de Cabeça e Pescoço/tratamento farmacológico , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/radioterapia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Modelos de Riscos Proporcionais , Doenças Raras/patologia , Doenças Raras/cirurgia , Estudos Retrospectivos , Programa de SEER , Taxa de SobrevidaRESUMO
This is the first report of a primary corneal fibrosarcoma in a canine. A 4-year-old male dog, Blue Heeler, owas examined because of a reddish nodular mass in the right eye. A complete ophthalmic exam was conducted, including slit-lamp biomicroscopy, applanation tonometry, fundoscopy, ocular and abdominal ultrasound, hemogram, serum biochemistry profile, and a thoracic X-ray. After the ophthalmic evaluation of the right eye, conjunctival hyperemia and the presence of a .5 cm diameter mass was found in the cornea. The diagnosis of fibrosarcoma was confirmed by histopathological analysis. The keratectomy showed satisfactory results in the removal of the corneal fibrosarcoma. The patient was followed up on for 18 months after the surgical procedure, and there were not any signs of local recurrence or metastasis.
Assuntos
Córnea/diagnóstico por imagem , Fibrossarcoma/patologia , Animais , Córnea/patologia , Córnea/cirurgia , Doenças do Cão , Cães , Fibrossarcoma/cirurgia , Fibrossarcoma/veterinária , Ceratectomia , Masculino , Tonometria Ocular/veterináriaRESUMO
Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype. The most common tumor locations are the deep soft tissue of extremities or trunks. We report a rare case of recurrent LGFMS in the inguinal region involving the scrotum and both testicles. A 38-year-old male patient reported a history of multiple nodular lesions in the left inguinal region accompanied by local inflammation. The patient was submitted for local resection of the lesion at our institution, with histopathological diagnosis of LGFMS. He missed his follow-up, returning with a large bulge in the left inguinal region involving the scrotum with signs of tissue necrosis and local purulent discharge. Surgical exploration was performed and the patient underwent tumor resection in the left inguinal region and the entire scrotum, with bilateral orchiectomy, with the margins enlarged to the right inguinal region and proximal surface of the penis. Local reconstruction was performed with a left fascia lata tensor muscle flap and ipsilateral thigh coverage using partial skin graft. On microscopic examination, the tumor showed spindle cells arranged in bundles, with abundant collagen and myxoid stroma with interspersed prominent vessels. The immunohistochemical study carried out showed immunoreactivity with Ki67 (<5%), immunonegativity with desmin and S100, confirming the diagnosis of LGFMS. Postoperative recovery was good and no recurrence was seen after two years. The patient is in good health, realizing multidisciplinary outpatient follow-up and performing continuous testosterone replacement. Surgical resection with negative margins for localized disease remains the standard treatment for LGFMS.
Assuntos
Fibrossarcoma/cirurgia , Escroto/patologia , Neoplasias de Tecidos Moles/cirurgia , Adulto , Fibrossarcoma/patologia , Humanos , Masculino , Escroto/cirurgia , Neoplasias de Tecidos Moles/patologiaRESUMO
Soft tissue tumors are not uncommon in childhood and comprise entities that range from common to very rare malignancies. Infantile fibrosarcoma (IFS) is a rare pediatric malignancy mainly seen in the first two years of life. The data about the incidence of infantile fibrosarcoma occurring in the neck in the Indian subcontinent is scarce. To the best of our knowledge, only one case of infant cervical IFS has been reported previously in the Indian subcontinent. We present another case of an eight-year-old male patient with a rapidly growing mass on the left side of the neck. He was successfully treated with a combined modality of surgery and chemotherapy with a good outcome. Among the soft tissue tumors of childhood, IFS is a rare entity. It has a good prognosis and lesser chance of distant metastasis as compared to adult fibrosarcoma. Though surgical excision is the mainstay of treatment, chemotherapy also has a significant role in the treatment of primary tumor and metastasis. We discuss the stated case to bring to the notice this uncommon cause, which can be considered as a differential diagnosis of upper cervical swellings. A better understanding of this entity would help in early diagnosis and aggressive treatment, reducing the overall morbidity and mortality.
Assuntos
Humanos , Masculino , Criança , Rabdomiossarcoma , Fibrossarcoma/patologia , Neoplasias de Cabeça e Pescoço , Neoplasias de Tecidos Moles/patologia , Diagnóstico DiferencialRESUMO
Feline injection site sarcoma (FISS) is a mesenchymal neoplasm with highly malignant characteristics. These tumours originate in anatomical sites where there has been previous parenteral administration of medicinal substances or implantation of medical devices. The aim of this study was to investigate the epidemiological and pathological features associated with FISS in the southern region of Brazil. The database of the Department of Veterinary Pathology of the Federal University of Rio Grande do Sul was searched for excisional and incisional biopsy samples compatible with FISS submitted between 2007 and 2017. Biopsy reports were reviewed and epidemiological information, including breed, age and sex of affected cats, as well as gross findings including anatomical location and size of the tumour and the presence of tissue invasion, were extracted. Eighty-nine samples were selected based on the established criteria. Most animals were of undefined breed and were female cats with a median age of 10 years. Grossly, 84.8% of the tumours were >2 cm in diameter. Regarding anatomical location, 34.9% of the tumours were located in the subcutaneous tissue of the thoracic wall, 29.2% in the flank, 21.3% in the interscapular region and 14.6% in the limbs. Histologically, the tumours originated in the subcutaneous tissue and were diagnosed as malignant mesenchymal neoplasms. Most were compatible with fibrosarcomas, but variants with features of pleomorphic sarcoma or chondrosarcoma were recognized. All tumours exhibited areas of necrosis and peripheral inflammatory infiltrate, composed predominantly of lymphocytes, plasma cells and macrophages. The results of this study suggest the need for dissemination of information on FISS epidemiology and guidelines for management of this tumour to veterinarians in the region.
Assuntos
Fibrossarcoma/veterinária , Reação no Local da Injeção/veterinária , Neoplasias de Tecidos Moles/veterinária , Animais , Brasil , Doenças do Gato/epidemiologia , Doenças do Gato/patologia , Gatos , Feminino , Fibrossarcoma/patologia , Reação no Local da Injeção/epidemiologia , Reação no Local da Injeção/patologia , Mesenquimoma/patologia , Mesenquimoma/veterinária , Estudos Retrospectivos , Neoplasias de Tecidos Moles/patologia , Vacinação/veterináriaRESUMO
INTRODUCTION AND OBJECTIVE: Oral benign neoplasms (OBNs) exhibit some features that can guide the professionals to the correct diagnosis and best treatment. Through retrospective studies, medical records can be reviewed to better describe a given population and, furthermore, help clinicians in routine practice. In this context, the objective of this paper was to analyze the cases of OBNs of an oral pathology referral department, from 2003 to 2017, in order to better understand their epidemiological and clinicopathological characteristics. METHODS: A total of 8355 histopathological reports were analyzed. Lesions diagnosed as OBNs were selected and the following variables were recorded: gender, age, histological type of the lesion, anatomical location, rate and pattern of growth, type of base, color, symptomatology and diagnostic hypotheses on clinical examination. RESULTS: OBNs represented 9.4% of all lesions diagnosed. The most frequent histopathological types were fibroma (39.9%), papilloma (22%), fibroblastoma (13.1%), lipoma (10.2%) and hemangioma (6.1%). Overall, most cases affected females (n=518; 65.6%) and in the fifth decade of life (n=148; 18.7%). The oral mucosa was the most common site (n=265; 33.5%). The most common features of each OBN were also highlighted. CONCLUSION: The most common OBNs were fibroma, papilloma, fibroblastoma, lipoma and hemangioma. Overall, the OBN presented common clinical features; however, in particular cases, there are some characteristics that can lead the professionals to the correct diagnosis. Nevertheless, in general, histopathological analysis must be performed to confirm diagnosis. Intraosseous tumors and large lesions may require imaging tests to help diagnosis.