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.     

Aggressive fibromatosis of the head and neck (desmoid tumours)

Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.     

头颈部韧带样瘤的治疗

目的 探讨头颈部韧带样瘤手术及放疗等治疗方法的效果.方法 回顾性分析复旦大学肿瘤医院头颈外科1987-2002年期间收治的44例头颈部韧带样瘤患者的临床资料和随访情况,评估外科治疗和放疗的疗效.结果 根据患者病情分为3组:单纯手术组15例,均为术后病理检查显微镜下证实切缘均为阴性者,随访12-156个月,局部复发率26.7%(4/15).手术+术后补充放疗12例,均为术后肿瘤残留患者,其中显微镜下病理检查切缘阳性5例,肉眼可见肿瘤残留7例,随访时间16～164个月,均无复发;外院手术后复发再放疗组17例,放疗后肿瘤残留率23.5%(4/17),随访时间15～136个月,局部复发率17.6%(3/17).所有患者未发现远处转移,也没有与此病相关的死亡.结论 对于头颈部韧带样瘤,外科手术难以切除彻底,应尽可能地保留患者的外形与功能,提高患者的生存质量.术后须补允放疗,能取得满意疗效.对于初次手术复发的患者,再行放疗,也能获得较佳预后.     

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。     

Primary haemangiopericytoma of the parapharyngeal space: an unusual tumour and review of the literature

Haemangiopericytoma is a rare soft tissue tumour, with great histological variability and unpredictable clinical and biological behaviour. The precise cell type origin is uncertain. One third of haemangiopericytomas occur in the head and neck area, but only a few cases have been reported regarding localization at the parapharyngeal space. Herewith, case is presented of a 54-year-old female, referred to our Department due to a parapharyngeal space tumour with non-specific imaging characteristics. The patient underwent radical excision of the tumour with a trans-cervical sub-mandibular approach. The histolopathologic examination revealed a neoplasm with the characteristic features of haemangiopericytoma. One year later, during the scheduled follow-up, the computerized tomography scan showed no evidence of recurrence or residual disease. The pre-operative evaluation of a haemangiopericytoma must include a thorough imaging evaluation with computerized tomography and magnetic resonance imaging, even if results may not be specific for haemangiopericytoma. Angiography and pre-operative embolization may be performed in cases of large tumours with significant vascularity. The treatment of choice is radical excision. The follow-up includes clinical evaluation every 6 months and annual magnetic resonance imaging for at least 3 years.     

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.     

Tumores primarios del espacio parafaríngeo. Nuestra experiencia en 51 pacientes

Introduction and objectivesTumours of the parapharyngeal space are rare, representing only 0.5 % of head and neck oncology. The aim of this study is to present our experience with the diagnosis and therapies for these tumours.Patients and methodThis study is a retrospective review of 51 patients diagnosed as having tumours of the parapharyngeal space and treated surgically between 1984 and 2006. Tumours originating in the deep lobe of parotid gland were excluded.ResultsSeventy percent of the parapharyngeal space neoplasms were benign and thirty percent malignant. Pleomorphic adenoma was the most common neoplasm (37 %), followed by miscellaneous benign tumours (34 %), paraganglioma (21 %), and neurogenic tumours (8 %). Surgery was chosen in all case, with the transcervical approach used in 32 cases, the cervical-transparotid approach in 13 cases, the transmandibular approach in 4 cases (with mandibulectomy in 1 patient), the transoral approach in 1 case, and the infratemporal fossa approach in 1 case. The most common complications were those deriving from damage to nerve structures.ConclusionsMost parapharyngeal space tumours can be removed surgically with a low rate of complications and recurrence. The transcervical approach is the most frequently used.     

头颈部韧带样瘤临床分析

目的 探讨少见的头颈部韧带样瘤的临床表现、诊断依据、治疗方法及预后,为临床医师正确认识该病,减少复发率提供帮助.方法 回顾性分析头颈部韧带样瘤4例,1例面颊部肿瘤位于右颞下窝及右咽旁间隙,1例左侧上齿槽肿瘤,侵犯同侧上颌窦及眶底,2例颈部肿瘤,分别位于右胸锁乳突肌中部后缘及右锁骨上窝,均采用手术切除.结果 术中见肿瘤实质性,呈浸润性生长,无包膜,可破坏周围骨质及肌肉.术后随访1～2年,1例颊部肿瘤及2例颈部肿瘤无复发,1例左上齿槽肿瘤于术后半年复发.结论 头颈部韧带样瘤为介于良性纤维母细胞瘤与纤维肉瘤间的交界性肿瘤,术前症状不典型,诊断主要依靠术后病理,彻底手术切除是降低复发的主要手段.     

Follicular dendritic cell tumor: an aggressive head and neck tumor

OBJECTIVE: To characterize the clinicopathologic features of head and neck follicular dendritic cell (FDC) tumor and report the experience of this entity at our institution. Study Design: Two case presentations are compared with a retrospective analysis of all published head and neck cases. SETTING: A tertiary academic medical center. RESULTS: Thirty four cases of FDC tumor of the head and neck cases have been published. Twenty five occurred in the cervical lymph nodes, 4 in the tonsils, 2 in the palate, 1 in the pharynx, 1 in the parapharyngeal region, and 1 in the thyroid gland. Patient ages ranged from 13 to 73 years (mean, 38), and there was a roughly equal number of men and women. Patients were treated with surgery (17), surgery and chemotherapy (8), and surgery and radiation (9). After the primary treatment, 12 patients had no evidence of disease, whereas 5 were incurable. Ten tumors recurred locally and 3 distally. Of these 13 patients who suffered recurrences, 4 had no evidence of disease after secondary treatment, 6 were alive with disease, and one was lost to follow up. Two patients died after recurrence. We add 2 unique cases to the 9 previously reported extranodal cases, 1 in the tonsil and 1 in the parotid gland. CONCLUSION: FDC tumor is a rare malignant neoplasm that can present in the head and neck region in both lymph nodes and extranodal sites. Because of their rarity, these tumors are probably underrecognized by both clinicians and pathologists. Distinct light microscopic, immunohistochemical, and ultrastructural features do exist, however, and are reviewed. Surgery has been the mainstay of treatment and should include diligent control of surgical margins. The role of adjuvant therapy remains controversial. Although originally considered to be a low-grade malignancy, our review suggests both high recurrence rates and metastatic potential. We believe that FDC tumor should be viewed and treated as a moderately aggressive head and neck tumor.     

Parapharyngeal space tumors. Our experience. I.P.O. Francisco Gentil,Lisbon

Primary parapharyngeal space tumours are rare, representing only a 0.5% of head and neck neoplasms. The authors report a case series review of 38 patients with parapharyngeal tumours who underwent surgical excision between 1975 and 1998. Twenty-six of them were female (68%) and twelve male (32%). Thirty-three tumours (87%) were benign and five (13%) were malignant being the Pleomorphic adenoma the most common neoplasm (39%). All patients were treated surgically: the trans-cervical approach was used in 19 cases, cervical-parotid in 5, the trans-parotid approach in 7 patients, transoral in 5, the cervical-parotid approach with mandibulectomy in 2 and the combined transoral-cervical approach in 1 case. Out of the 33 patients with benign neoplasms, 1 (a pleomorphic adenoma treated through a transoral approach) had a recurrence. Amongst the 5 with malignant disease, recurrence or persistent local tumour was seen in 4 cases; and of these, 3 with persistent local tumour after incomplete excision died.     

Myxoma of the maxilla

Myxomas are benign connective tissue tumours, notorious for their aggressive behaviour and stubborn tendency of recurrence. These tumours are extremely rare in the head and neck region. One case of myxoma of the maxilla is being reported here.     

Aggressive fibromatosis of the head and neck in the pediatric population

Background

Aggressive fibromatosis, previously referred to as desmoid tumor, is a rare tumor. It is classified as benign, however, has a high potential for local invasion and recurrence. It is most commonly found in the abdomen, with an origin in the head and neck accounting for less than a quarter of the total cases.

Methods

Literature review using PubMed and OVID and a combination of the words, “desmoid”, “fibromatosis”, “pediatric”, “children”, and “head and neck”.

Results

Annual occurrence is 0.2 – 0.4 per 100,000, with origins in the head and neck accounting for 10–25%. Since 1954, 97 cases of pediatric head and neck fibromatosis were reported. The age range was from birth to 16 years, with the average being 4 years and 5 months old. The overwhelming majority of tumors were of the mandible (38%). 74% patients underwent a large resection as the primary treatment modality. The most common treatment complication was tumor recurrence (16%).

Conclusions

Fibromatosis is a rare tumor of muscoloaponeurotic origin. Surgery is the mainstay of treatment; however recurrence rates are high, even despite negative surgical margins.     

Extramuscular soft tissue myxoma of the lateral neck

Myxomas are rare benign connective tissue tumours of unclear histogenesis. In this case a lateral neck tumour initially behaved, both clinically and radiographically as a lipoma, and was therefore managed conservatively. Subsequently the lesion caused pressure symptoms and therefore a surgical approach was taken with subsequent histology confirming a myxoma. Only five cases of soft tissue myxoma of the lateral neck have been reported in the English literature of which two were extramuscular. This is the largest reported myxoma of the head and neck region and was treated with simple enucleation. There has been no evidence of recurrence five years after surgery.     

Extraskeletal Ewing's Sarcoma of parapharyngeal space

Extraskeletal Ewing's Sarcoma (EES) of head and neck region, is a rare soft tissue tumour morphologically similar to the commoner Ewing's Sarcoma arising from bone. We report a case of EES in parapharyngeal space in a 6-year-old male; who presented with stridor. CT scan of neck showed soft tissue mass in right parapharyngeal space with intraorbital and intracranial extention. Histopathological examination showed round cell tumour and immunohistochemistry was positive for CD99 and Vimentin. The patient responded very well to Concurrent Chemoradiation. An extensive review of English literature, to the best of our knowledge, did not reveal any previous case of EES in parapharyngeal space in children; although one similar case in a 53-year male is reported.     

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。     

Malignant triton tumour of the parapharyngeal space

We report the clinical course in a 25-year-old male patient of neurofibromatosis with malignant triton tumour (MTT). Triton tumour is a peripheral nerve sheath tumour with rhabdomyoblastic differentiation. This is relatively rare tumour of head and neck region and only 26 cases have been reported to date. The present case is the first reported MTT of parapharyngeal space, one of the most aggressive course among all head and neck MTTs, resulting in the death of the patient within three months following surgical resection. The aggressive nature of this tumour necessitates adjuvant therapeutic measures in addition to radical surgery in order to obtain better survival rates.     

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.     

Desmoid tumour of the neck: complete regression following radiation therapy

Desmoid tumours are uncommon benign neoplasms. They arise from fascial and musculoaponeurotic tissues and are locally infiltrative, resulting in a high rate of local recurrence following surgical resection. They present difficult loco-regional control. The location and extent of the tumour, as well as the potential for significant morbidity and mortality, dictate the most appropriate therapeutic option. Complete surgical extirpation is the optimal treatment for primary and recurrent desmoid tumours. Radiotherapy is indicated in incompletely excised or recurrent tumours. Radiation as a primary treatment is seldom recommended. A case of desmoid tumour of the neck, primarily treated with radical radiation, is described and relevant literature reviewed.     

Successful treatment of an extra-abdominal fibromatosis (desmoid tumor) arising from the prevertebral fascia of the neck

We describe the successful treatment of a fibromatosis (desmoid tumor) arising from the prevertebral fascia of the neck. Total resection with wide margins is reportedly the best treatment for this kind of tumor. However, the anatomy of the head and neck makes such resection difficult. In this case, we were unable to completely remove the tumor because it was large and located close to the cervical vertebrae, common carotid artery and internal jugular vein. Incomplete resection is known to result in higher tumor recurrence than complete resection. In addition, the recurrence or progression of a tumor in the head or neck region is known to cause mortality by compression of the airway or major blood vessels. On the basis of reports that irradiation is effective treatment for this kind of tumor, we administered 30-Gy irradiation to the affected area. This therapy was very effective and no sign of recurrence was seen for 2 years after irradiation. We found that function-sparing resection plus postoperative radiotherapy is an effective treatment for advanced fibromatosis in the head and neck regions with proximity to or involvement with vital structures.     

Synovial sarcomas of the neck

The synovial sarcoma is a mesenchymal tumour that is very rare in the head and neck region. This is a report on two cases. In a 30-year old patient the tumour arose near the thyroid gland and the hypopharynx. He died in spite of surgical removal, radiotherapy and chemotherapy, one year after the first symptoms. An 8-year old boy was affected by a synovial sarcoma near the hyoid bone and is without recurrence after surgical removal, radiotherapy and chemotherapy. Subsequently, the main diagnostic characteristics of these tumours are discussed. A better prognosis of the tumours associated with the hyoid bone is a remarkable fact.