儿童头颈部侵袭性纤维瘤病(附3例报告)

目的：探讨儿童头颈部侵袭性纤维瘤病的治疗方法。方法：回顾性分析3例头颈部侵袭性纤维瘤病患儿的临床资料。结果：3例患儿均经手术切除肿瘤,病理明确,随访过程中无复发。结论：儿童侵袭性纤维瘤病局部呈浸润性生长,手术彻底切除是主要的治疗方法,但由于儿童生长发育问题很难做到彻底切除,由于位于头颈部,易造成面容破坏和畸形。     

Aggressive fibromatosis of the neck treated with a combination of chemotherapy and indomethacin

Aggressive fibromatosis (desmoid tumor) of the neck is rare. When feasible, surgery is the best treatment option. However, complete excision with negative margins is not possible in most cases because of the involvement of vascular and nervous structures. Also, surgery results in poor functional and aesthetic outcomes. Sometimes debulking surgery with positive margins is performed, but the anatomy of the neck is a challenge for oncologic surgeons, and recurrences are not uncommon. Radiotherapy is seldom employed for the same reasons. On the other hand, systemic treatment with chemotherapy, hormone therapy, and noncytotoxic agents such as nonsteroidal anti-inflammatory drugs (NSAIDs) has been used with good results. We report a case of inoperable aggressive fibromatosis of the neck that was successfully treated for about 21 months with a combination of chemotherapy and the NSAID indomethacin. As far as we know, this is the first reported use of a combination of chemotherapy and an anti-inflammatory drug in the treatment of aggressive fibromatosis of the neck. We also review the literature on cases of aggressive fibromatosis of the neck that have been reported over the past 12 years.     

手术联合放疗治疗头颈部侵袭性纤维瘤病12例报道及文献复习

目的探讨头颈部侵袭性纤维瘤病最佳的治疗方法。方法对12例头颈部侵袭性纤维瘤病进行手术治疗,其中7例术后辅助放射治疗。结果所有患者术后随访5～15年,7例行肿瘤切除加术后放疗者无复发,5例行单纯肿瘤广泛切除者术后7～18个月复发;总复发率为41.7%。术中切缘行病理检查,切缘阴性者复发率为14.3%。结论术中确定阴性切缘可以降低头颈部侵袭性纤维瘤病复发率,但头颈部病变多难以获得阴性切缘,局部切除后易复发,术后放疗能降低复发率。     

头颈部侵袭性纤维瘤病2例及文献复习

目的:探讨头颈部侵袭性纤维瘤病的临床表现、病理特征、治疗方式及预后,为临床医生正确诊治及降低该病复发率提供帮助。方法:回顾性分析2例头颈部侵袭性纤维瘤病的临床资料并复习相关文献。结果:侵袭性纤维瘤病以浸润性生长、局部易复发及无远处转移为特点。病理学表现为成纤维细胞单克隆增生,由分化良好的成纤维细胞和肌成纤维细胞组成,无恶性细胞学特征和有丝分裂相。本组患者中1例第1次手术后复发,再次手术后随访6个月无复发;另1例手术后随访6个月未复发。结论:头颈部侵袭性纤维瘤病症状无特异性,诊断主要依靠术后病理,彻底手术切除是治疗并减少复发的主要手段。对于复发或难以完整切除的患者,可配合放化疗。     

Desmoid tumor of the parotid gland

INTRODUCTION: Desmoid tumor is a benign microscopic tumor that belongs to the group of the deep fibromatosis. It usually arises from facial or musculoaponeurotic structures in the abdomen but rarely is located in the head or neck. Locally, it is characterized by a massive invasion with strong potential for recurrence.CASE REPORT: A 37-year-old woman was hospitalized for a swelling of the left parotid gland region which had progressed for eight years. Physical examination revealed a tumor in the parotid gland, which was hard and adherent deeply, measuring 4 cm/3 cm and sensitive to palpation; without satellite nodes nor facial paralysis. The treatment consisted in a superficial parotidectomy. Pathology was in favor of a desmoid tumor. After an 18-month follow-up, the patient is healthy without any sign of recurrence.DISCUSSION: Desmoid tumors are deep fibromatosis characterized by their slow growth and especially by considerable infiltration of the adjacent structures but without potential for metastasis. Although very rare, the cervical localizations are especially aggressive. This clinical case illustrates the clinical features of this rare affection and offers matter for discussion of therapeutic difficulties.     

Syndromic gingival fibromatosis

INTRODUCTION: Gingival fibromatosis is an inherited disease of the gingival tissue causing proliferative fibrous lesions occurring alone or as one feature of a more widespread syndrome.OBSERVATION: A 26-year-old girl consulted the oral surgery department for a functional problem due to the presence of two tumor masses located in the palate. The histological diagnosis was gingival fibromatosis. The presence of skeletal disorders in this patient with gingival fibromatosis suggested the diagnosis of Zimmermann Laband syndrome.DISCUSSION: Gingival fibromatosis is a rare condition characterized by a gingival hypertrophy. It is transmitted by autosomal dominant or recessive inheritance. The oral examination and histology provides the diagnosis. The treatment consistes of surgical removal of the hyperplasic fibrous tissue. Prognosis is dominated by the risk of recurrence which is frequent. Generally, gingival fibromatosis is isolated. In rare cases, it can be associated with symptoms sugges-ting a syndromic diagnosis such as Zimmermann Laband syndrome. In addition to gingival hypertrophy, skeletal anomalies can involve the fingers, the toenails, the thorax or the vertebrae. Splenomegaly and joint hyperextensibility can also be found.     

Mandibular Langerhans cell histiocytosis

INTRODUCTION: Langerhan's cell histiocytosis is defined as an abnormal proliferation of Langerhans cells in various organs and tissues (bone, skin, lymph nodes...). Among facial locations, mandibular involvement is the most frequent and occurs in young people less than 20 years. The aim of this report is to review the characteristic features of this disease, the current means of diagnosis and treatment through two case reports.CASES: Two male patients aged 10 and 55 years respectively presented with single mandibular location of Langerhans cell histiocytosis. The diagnosis was confirmed by histological examination of the biopsy specimen. The first patient underwent surgical resection of the tumor. No recurrence was observed. In the second patient, the postoperative course was marked by the appearance of an inflammatory phenomenon in the adjacent skin and mucosa, successfully treated by intralesional corticosteroid therapy.DISCUSSION: Langerhan's cell histiocytosis is a rare disease. The mandibular location has variable clinical expressions which can vary from dental mobility to tumor development in the gingiva. The radiological appearance is also variable; histological proof is required for diagnosis. Treatment depends on the number and locations of the lesions. In case of a single location, the treatment options may vary from a "wait and see" attitude to a non aggressive surgery, with a possible local corticosteroid therapy or radiothe-rapy. For multiples locations, chemotherapy is indicated.     

头颈部侵袭性纤维瘤病(3例报告及文献复习)

目的：探讨一种头颈部少见肿瘤--侵袭性纤维瘤病的临床表现、病理特点、治疗选择及转归。方法：报告3例头颈部侵袭性纤维瘤病的临床资料并复习相关文献。结果：①侵袭性纤维瘤病是一种纤维增生性良性或低度恶性肿瘤生物学呈局部浸润性和破坏性生长；②病理学表现为细胞中心和胶原周围成纤维细胞单克隆增生，由分化良好的成纤维细胞和肌成纤维细胞组成，缺乏恶性的细胞学特征和有丝分裂相，电镜示丰富的胶原网包绕多形态细胞增生，免疫组化波形蛋白和肌动蛋白阳性；③手术是主要治疗措施，多数病例需多次手术，术后复发率可高达70％，常需配合放射治疗和化学治疗；④1例接受3次手术仍复发，另2例手术后随访3-6个月未复发。结论：侵袭性纤维瘤病是一种少见的纤维增生性肿瘤，头颈部少见；以手术治疗为主，有高度复发倾向，但预后较好；对于复发或难以完整切除的病例，常需配合放射治疗和化学治疗。     

Desmoid tumours of the head and neck: A case report

IntroductionDesmoid tumours of the head and neck, also known as fibromatosis, are rare, locally invasive benign tumours with high recurrence rate, causing considerable morbidity. Complete surgical excision of desmoid tumours is considered to be the only effective treatment.Case reportWe present a case of fibromatosis of the right posterolateral region of the neck in a 56-year-old woman who presented with right neck mass. The patient underwent complete excision of the tumour with no adjuvant therapy. No recurrence or neurological deficit was observed 2 years after surgery.ConclusionAlthough desmoid tumour is a benign neoplasm with no metastatic potential, treatment is challenging due to its aggressive, infiltrative behaviour with a tendency to recur.     

Multiple metastases of a mandibular ameloblastoma

INTRODUCTION: Ameloblastoma is a benign odontogenic tumor which can be locally aggressive and invasive. Metastases are rare but possible and must be considered as a malignant form of the tumor. OBSERVATION: A 50-year-old woman presented a jugal metastasis of a mandibular ameloblastom which had been treated several times 28 years earlier. Six months after resection of the metastasis, the patient developed multiple cervical node metastases which were removed by radical curettage. Five months later, new metastases developed on the scalp and three months later a voluminous metastasis involving the right hemiface extended to the base of the skull. Surgical resection was only partial followed by radiotherapy. Ten months later the outcome was favorable. DISCUSSION: The absence of any histological sign of malignity in the primary tumor and in the metastases, as observed in our patient, is remarkable. Metastases generally develop in the lung (61-80% of cases). Metastases to the scalp have never been described. The time to development of a metastasis is generally very long and metastases usually remain asymptomatic. Progression is very slow, like for the primary tumor. Several factors predictive of metastasis have been described: female gender, age at onset of primary tumor (2nd to 3rd decade) and multiple local recurrences. There is no standard treatment for metastases. Chemotherapy is not effective. Radiotherapy may be effective, particularly when lesions are not accessible to surgery. Surgical resection remains the treatment of choice.     

Diagnosis and therapy of aggressive fibromatosis (extra-abdominal desmoid) in the head and neck area

Desmoid tumours (extraabdominal desmoids, aggressive fibromatosis) are rare connective tissue tumours, that grow infiltratively and destructively. The analysis of the course of disease in three patients suffering from desmoid tumours in the head and neck region (regio submandibularis, regio parotidea, parapharyngeal space) confirmed the data in literature that painless swelling and dysfunction are the first non-specific signs of the disease. Morphologically reactive fibromatosis and fibrosarcoma should be considered (amongst others) in differential diagnosis. CT and MR scans are useful in determining the extent of the tumours and help to distinguish the tumour from nerves, vessels and bone. Surgery is the therapy of choice in the head and neck region. Primary surgery was performed in our patients. Due to recurrences two of our patients have been operated on several times including major plastic reconstructive surgery. One patient died three and a half years after primary treatment due to sarcomatous degeneration. A large parapharyngeal recurrence was successfully reduced by percutaneous radiation therapy in our second patient. Follow-up of our third patient was NAD 15 months after resection of a large parapharyngeal/mediastinal process including blocking of two cervical vertebrae. Summing up, desmoid tumours are rare connective tissue tumours in the head and neck region with a destructive biological behaviour similar to malignant tumours and a high recurrence rate. Surgery is the treatment of choice. In recurrences or if the tumour is not properly resectable, radiation must be considered, whereas the chemotherapeutical approach is not yet well established.     

Aggressive fibromatosis of the parapharyngeal space: two cases and treatment recommendations

Aggressive fibromatosis is an uncommon tumor that is locally aggressive but not malignant. Therefore, its classification falls between the benign and malignant neoplasms of soft-tissue origin. In the past, these lesions were misdiagnosed as low-grade fibrosarcomas. Otolaryngologists should be familiar with these lesions because as many as 15% of them occur in the head and neck. The treatment of choice is wide surgical excision, which is often difficult. Postexcision recurrence rates are high. Nonsurgical treatment includes radiation and chemotherapy, both of which are usually reserved for recurrences. We describe two cases of aggressive fibromatosis of the parapharyngeal space, and we review the available treatment options.     

Algorithm for the treatment of ameloblastoma in Tunisia

INTRODUCTION: Ameloblastoma is a benign but locally aggressive and infiltrative odontogenic neoplasm. Numerous methods of treatment have been proposed but the latest studies showed that a radical treatment is the recommended protocol for the surgical management of ameloblastomas. The purpose of this study was to review the clinical features and surgical treatment of ameloblastomas treated in our department and to propose an algorithm for the treatment adapted to our country. PATIENTS AND METHODS: Data was collected from the records of patients treated at our Surgery Unit between 1993 and 2004. Data with respect to the patients' ages, sex, tumor locations, and surgical treatment history, as well as radiographic findings and number of recurrences, was analyzed. RESULT: 8 male and 5 female patients were included in the study. Sixty-two percent of ameloblastomas were located in the mandibular angle. Swelling was the most common symptom and was experienced by 85% of the patients. A radiographic assessment of the tumors showed that 85% were multicystic. Conservative surgery was used in 92% of the cases. The 8 patients followed-up all presented with recurrence. The other 5 patients were lost to follow-up. A radical treatment was used for 6 of the recurrent ameloblatomas with immediate iliac bone reconstruction. One patient refused radical treatment, he benefited from a conservative treatment, and the other one was lost to follow-up. DISCUSSION: This study was characterized by the preponderance of the conservative treatment, the non-specification of histological types, systematic relapse, and the significant number of lost to follow-up. We recommend significant modifications in the management of ameloblatomas in Tunisia, based on radical surgical treatment. This suggests implementing further training of operators (free flaps) and pathologists (histochemistry), as well as a better education and information of patients.     

Fibromatosis of the infratemporal fossa extending to the parapharyngeal space accompanied by trismus

Fibromatosis is a tumor of fibroblastic origin that may arise in any musculoaponeurotic structure. Although fibromatosis is histologically benign, it has a tendency to invade surrounding tissues and often recur locally after treatment. A 34-year-old female patient was presented with pain in the left buccal region, left loss of visual acuity, and trismus. Computed tomographic scans revealed a large mass in the infratemporal fossa and parapharyngeal space. An open biopsy was performed and the pathological diagnosis was a fibromatosis. Surgical treatment for her trismus was performed simultaneously.     

Synovial sarcoma of the mandible in children. Apropos of a case

Synovialosarcoma is an aggressive malignant soft-tissue tumor. It's a mesenchymal tumor rare in the cephalic region in the children. Its occurs most frequently in the extremities, in the adolescents and young adults between 15 and 40 years. His treatment are principally a radical surgical excision. We report the case of a 10 year old boy who had a mandibular tumor developing in the first premolar area and invading the submandibular region. The histologic diagnosis was biphasic synovialosarcoma with epithelial predominance. The staging showed a stage II tumor (5 cm) of the submandibular region with invading the mouth floor and the mandible. After the failure of the polychemotherapy we had performed a radical surgical excision with a functional cervical lymphadenectomy. The tumor was excised in one piece with the horizontal part of the left hemimandible. The treatment was completed by radiotherapy. In a second time a reconstructive surgery was performed with a fibula free flap. The result at one year show a good local control and a perfect esthetic and functional result of the mandible. CONCLUSION: Synovialosarcoma is a very aggressive malignant soft-tissue tumor with a high metastatic risk. Management must be rapid as soon as the diagnosis is made. Surgical excision is the main treatment in association with the chemotherapy and radiotherapy.     

Differentiating between congenital rhabdomyosarcoma versus fibromatosis of the pediatric tongue

Congenital rhabdomyosarcoma of the tongue is exceedingly rare. Fibromatosis of the tongue is also rare, and very difficult to distinguish from the spindle cell variant of rhabdomyosarcoma. Both appear histologically as spindle neoplasms replacing normal striated musculature of the tongue. The treatment protocol for the former has been developed by the Intergroup Rhabdomyosarcoma Studies (IRS) I-IV and requires surgery, radiation, and chemotherapy. For fibromatosis, complete surgical excision is usually adequate without additional therapy, although some cases of aggressive fibromatosis also require chemotherapy. With significant differences in appropriate treatment and prognosis, each entity must not be mistaken for the other. We review the differences in radiologic, histologic, and immunohistochemical (IHC) features of both entities.     

Desmoid-type infantile fibromatosis in the mandible: a case report

A 3-year-old boy with desmoid-type infantile fibromatosis arose in the mandible was reported. He was referred to our hospital because of suspected malignant bone tumor of the mandible. Histological examination of an open biopsy specimen was performed followed by tumor resection with marginal mandibulectomy and reconstruction by iliac bone grafting, which caused no functional complications nor mandibular deformity. To treat tumors in the facial skeleton, the surgical procedure should be planned based on the histological diagnosis in order to determine the proper area of resection to prevent functional or cosmetic complications. Especially in children, attention should be taken for benign but clinically resembling malignant rare diseases such as desmoid-type infantile fibromatosis.     

Gastric scirrhous carcinoma as a rare cause of aggressive fibromatosis in the neck

Aggressive fibromatosis is characterized by locally aggressive proliferation of fibroblasts and occasionally occurs in the head and neck. Although the etiology of this disease is still controversial, recent studies have shown that high levels of growth factors are found in these lesions. This article reports a case of 51-year-old woman with aggressive fibromatosis in the neck and inguinal region bilaterally. Gastric fiberscopic examination revealed a complication of advanced gastric scirrhous carcinoma. There were remarkably high levels of basic fibroblast growth factor (bFGF) and platelet-derived growth factor (PDGF) in the serum. Postmortem autopsy examination was performed and scattered gastric carcinoma cells were observed in the fibromatosis lesions. Immunohistological staining showed positive expression of bFGF in the fibromatoses and stomach. We concluded that this was a rare case in which bFGF and PDGF released from gastric carcinoma cells caused aggressive fibromatosis by promoting unregulated proliferation of fibroblasts and collagen production locally.     

Odontogenic myxoma of the maxillary

INTRODUCTION: Odontogenic myxoma is a rare benign tumor that arises from the mesenchymal portion of the tooth germ. It has a va-riable non-specific clinical and radiological appearance, and may be confused with other lesions such as ameloblastoma. CASE REPORT: We describe the case of a young, African male patient with a large odontogenic myxoma of the maxillary. The patient presented a painless firm swelling of the left maxillary. The radiograph showed a radiolucent lesion that was compatible with several diagnoses. DISCUSSION: The diagnosis was based on clinical, radiographic and histopathologic features. This tumor is locally aggressive, inducing important facial deformation and tooth displacement. Radical treatment with surgery or prosthesis is needed due to the high rate of recurrence.     

Pediatric mandibular myofibromatosis

INTRODUCTION: Pediatric myofibromatosis is a rare tumor in neonates and children. Two forms are described, solitary and multicentric, the solitary type is more common and is localized mainly on the head and the neck, mandible involvement is rare. The aim of this article was to report the anatomoclinical and therapeutic features of this pediatric tumor in a case as well as its follow-up. CASE REPORT: A 10-year-old girl was brought to consultation for a lower left gingival swelling 5 cm in diameter, forming a unit with the mandibular bone. The volume had gradually increased over the last 12 months. Imagery revealed the presence of an osteolytic tumor benign in aspect, but locally aggressive. Conservative surgery was performed. The diagnosis of pediatric myofibromatosis was confirmed. Evolution was excellent and after three years of follow-up, there was no evidence of relapse. DISCUSSION: Pediatric myofibromatosis often presents as a painless, well-circumscribed, solid nodule. Imagery is very useful to assess lesion extension and for the therapeutic follow-up. The diagnosis is made on anatomopathological findings and immunohistochemical assessment. The treatment of the solitary myofibromatosis is primarily surgical and its prognosis is excellent contrary to the multicentric form.