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.     

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.     

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.     

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。     

Particularidades del abordaje transcanal endoscópico de los tumores benignos de oído medio en adultos

Background and objectiveMinimally invasive surgery of benign middle ear tumours is possible by using the endoscope. The optimal lighting and the broadest vision it offers, allow a transcanal approach to these rare tumours. The objective of this work is to summarize its key points through a case series.Materials and methodsRetrospective study of benign middle ear tumours that underwent exclusive endoscopic surgery in a third-level adult university hospital between June 2018 and June 2020. Postoperative follow-up was performed by otoendoscopy and audiometry.ResultsSix patients underwent surgery during the study period. Five patients were female and one male, with an average age of 57.8 years (± 21.9). Four tumours were in the left ear and 2 in the right ear. These included 4 tympanic paragangliomas (3 type I and one type II), a chorda tympani neuroma, and a congenital cholesteatoma. There were no serious postoperative complications. At present, no tumour recurrence has been found in either case, with a minimum follow-up of 7 months.ConclusionsThe present study adds evidence on the safety and efficacy of endoscopic transcanal ear surgery, as a minimally invasive technique, for the treatment of benign middle ear tumours confined to the tympanic cavity.     

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.     

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.     

Fibroma of the tendon sheath of the neck

IntroductionFibroma of the tendon sheath (FTS) is a rare benign tumour typically occurring in the extremities, but very rarely involving in the neck.Case reportA 22-year-old male presented with a large painless mass of the right oropharynx. Magnetic resonance imaging (MRI) showed a well-circumscribed 7 cm lesion in the right prestyloid space. The lesion was completely removed surgically. Histopathological examination revealed a fibroma of the tendon sheath of the stylohyoid muscle.DiscussionThese tumours generally arise in the extremities of adults. To our knowledge, this is the first reported case of FTS in the neck.     

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.     

Fibromatosis of Thyroid Gland-A Case Report

Fibromatosis covers a broad group of benign fibrous tissue proliferations of varied microscopic appearances that are intermediate in their biological behaviour between bengin fibrous tumour and fibrosarcoma, Extra abdominal fibromatosis in the neck occassionally cause serious sequellae when aggressive invasion of vital structure occurs. However, the principle of wide excision is difficult to apply in some patients because of the important organs surrounding the tumour. We describe a primary spindle cell tumour of thyroid that raised initial diagnostic problems and was eventually diagnosed as fibromatosis of thyroid gland.     

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.     

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.     

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.     

Intralabyrinthine sporadic endolymphatic sac tumour

IntroductionEndolymphatic sac tumours are benign, slowly growing tumours that invade the temporal bone, and present clinically in the form of unilateral hearing loss. They can be sporadic or occur in the context of Von Hippel-Lindau disease (VHL).Case summaryThe authors report a case of endolymphatic sac tumour arising in the utricle presenting histological and immunohistochemical features corresponding to endolymphatic sac tumour in a patient without VHL.DiscussionEndolymphatic sac tumours invade the posterior part of the petrous temporal bone. According to two studies concerning patients with Von Hippel-Lindau disease, endolymphatic sac tumours arise from the endolymphatic duct. This case of intralabyrinthine sporadic endolymphatic sac tumour supports this hypothesis for sporadic forms, indicating the need for labyrinthectomy associated with tumour resection to avoid recurrence.     

Thirteen years' experience with superficial partial parotidectomy as treatment for benign parotid tumours

IntroductionMost authors agree that surgery is the treatment of choice for benign tumours of the parotid gland. However, the best surgical technique and the extent of surgery remain controversial. This study attempts to establish whether the implementation of a partial superficial parotidectomy (PSP) is appropriate for the treatment of benign parotid gland tumours.Material and methodsWe selected 63 patients with benign parotid gland surgery, of whom 43 had a pleomorphic adenoma and 20, a Warthin tumour. Of this group of 63 patients, 6 could not be included. We consequently studied 57 patients, 41 of them diagnosed as pleomorphic adenoma and 16, as Warthin tumours. In all of them, a PSP was performed without intraoperative monitoring of the facial nerve.ResultsTransient facial nerve paralysis, 14 patients (24.5%). Ten cases were resolved within the first month after surgery and 4 before the third month, after indicating facial physiotherapy. One patient (1.7%) had a permanent difficulty in keeping one side of the lower lip aligned under pressure-mobility, without altering commissure mobility. None of the patients studied had a recurrence (control, 3-13 years).ConclusionsAlthough PSP is a technique with a few complications, it has a recurrence rate comparable to or lower than other techniques used for the treatment of pleomorphic adenomas or Warthin parotid tumours. Intraoperative facial nerve monitoring can be helpful during surgery. The lack of monitoring would not be considered a contraindication for surgery.     

Synovial sarcoma of the ethmoid sinus

IntroductionSynovial sarcoma is a malignant mesenchymal tumour typically occurring in the extremities. Its occurrence in the head and neck region, particularly in the paranasal sinuses is extremely rare.Case reportHere, we report a case of primary synovial sarcoma of the ethmoid sinus in a 36-year-old female and review the literature of synovial sarcomas arising from the ethmoid sinus. Histopathology was essential in confirming the diagnosis. The patient underwent endoscopic excision of the tumour followed by postoperative radiotherapy.DiscussionAs per our knowledge, only 2 cases of ethmoid sinus synovial sarcoma have been reported in the English literature till date. Although synovial sarcomas rarely involve the paranasal sinuses, they should be included in the differential diagnosis of paranasal sinus tumours. The accepted treatment modality is wide local excision followed by post operative radiotherapy.     

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.     

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.     

Masson tumor as a neck mass

IntroductionA Masson's tumor is a benign, intravascular tumor, typically located in the fingers. Histologically, a Masson's tumor is characterized by an intravascular endothelial papillary projection associated with thrombi, without atypia or necrosis. The CD 31 and CD 34 stains are the most efficient immunohistological markers to identify the vascular origin.Case summaryA 54-year-old man presented with a right lateral neck mass for 3 weeks. The CT scan of the neck showed an oval, well-circumscribed cystic mass, measuring 20 mm × 9 mm, situated over the right sternocleidomastoid muscle, with a peripheral vessel draining into the right anterior jugular vein. The mass was excised surgically, and a pathological report indicated a thrombosed material inside the lumen of a small vein with a differentiated papillary structure; neither nuclear atypia nor necrosis were seen. The patient had an uncomplicated recovery and completed 8 months of follow-up appointments without any evidence of recurrence.DiscussionIntravascular papillary endothelial hyperplasia (IPEH) can be differentiated from angiosarcoma by lack of extension to the perivascular tissue and absence of necrosis and atypia. The pathogenesis is still unknown; surgical excision is the method of treatment. This is an extremely rare presentation of Masson's tumor.     

Malignant eccrine hidradenoma of neck causing acute heart failure

Tumours of eccrine sweat glands are uncommon, with complex classification and different terms used even for the same tumour. Therefore, for practical purposes, it may be sufficient to differentiate between benign and malignant. Malignant eccrine hidradenoma has a predilection for head and neck, with high incidence of recurrence following surgical excision and also regional and distant metastases. We describe a case, which presented as a medical emergency with symptoms of severe anaemia and acute heart failure secondary to intermittent bleeding from a huge ulcerative neck lesion, which was subsequently diagnosed as eccrine hidradenocarcinoma. The tumour was successfully treated with complete surgical excision and reconstruction followed by radiotherapy. Two years postoperatively, the patient is very well with no sign of recurrence.