马德龙病1例报告

马德龙病又称为多发性对称性脂肪瘤病,是一类罕见的因脂肪代谢障碍引起的疾病,多发于30~60岁的中老年男性,主要病变特征是大量无包膜脂肪组织团块呈弥漫性、对称性聚积在颈、项、肩以及其他躯干部位的皮下组织内,目前对于该病国际上尚无标准有效的治疗方法。本文报告我科收治的1例马德龙病例,并基于现有文献资料,对马德龙病的病因、临床分型、特征性表现以及诊断、鉴别诊断和治疗等进行讨论。     

Madelung's disease (benign symmetric lipomatosis)

Madelung's disease, benign symmetric lipomatosis, is a rare disease of undetermined cause characterized by symmetric deposits of diffuse adipose tissue on the cheeks, the neck, and the upper trunk. This article outlines our experience with four patients and presents a case report of a specifically remarkable affectation that agrees with previous data linking male alcoholics with this disease. The treatment in all cases was palliative surgical removal of excess fat from the neck and paracervical regions.     

Facelift- and circum-occipital incision placement for fat extirpation of the neck in Madelung's disease – A two-case report

Benign symmetric lipomatosis (Madelung's disease) is a rare disorder of fat metabolism that is characterized by progressive symmetrical formation of unencapsulated and painless excess fat masses around the neck and trunk that result in cosmetic disfiguration and functional impairment. Since the disorder is incompletely understood and causal therapy is unavailable, surgical removal of fatty masses is the mainstay of treatment. In this paper the authors describe their use of the classical facelift incision placement as well as a horizontal circum-occipital incision to approach and excise excess fat of the anterior and posterior neck in two patients. This method yielded satisfying results with a combination of good access to fat masses, smooth trimming and redraping of redundant skin, in addition to fairly inconspicuous scarring post-operatively. After removal of 1.5 kg of fat from each patient and a period of uncomplicated wound healing, both patients showed no signs of relapsing fatty growth. Although more challenging than most conventional approaches, the authors' technique has shown good outcomes in those treated with this condition.     

Madelung病:病例报告与文献复习

回顾性分析1 例Madelung病患者的病例资料,讨论此病病因,临床表现和治疗方法.该病病因不清,多发男性,肿块对称性地分布在头、颈及上躯干皮下的多个部位,呈现"马颈"或"驼峰"样外形,组织病理学表现为脂肪样组织沉积.目前手术是主要治疗手段,其预后较好.     

Long-term results following surgical treatment of benign symmetric lipomatosis (BSL)

Benign symmetric lipomatosis is a rare disease. Its main characteristic is the symmetric deposition of fat, in an unencapsulated form, at typical sites in the body, but primarily in the head and neck region. Its aetiopathogenesis is unknown. A disturbance of lipid metabolism is involved, and there is a proven connection with chronic alcoholism. Problems with the differential diagnosis of the characteristic clinical picture arise from the fact that this disease is not widely recognized. Over a period of 30 years, the authors treated 31 patients with benign symmetric lipomatosis. They review the pertinent literature and report retrospectively on the fates of the 11 surviving patients.     

Bilateral asymmetric tongue classic lipomas

Lipomas are most common benign mensenchymal neoplasm composed of mature adipocytes. But they are relatively infrequent on the oral and maxillofacial regions. In the present report, we report a rare case of bilateral involvement classic lipoma of the tongue. The patient was a 71-year-old man with asymmetric soft tissue nodules in both lateral borders of the tongue. The magnetic resonance imaging T1- and T2-weighted images demonstrated homogeneous high intensities similar to that of normal subcutaneous fat. The lesions were hypointense on the fat-suppressed T1 image. The tumors were resected surgically, and they exhibited the histopathologic features of classic lipomas, composed of mature fat cells. This suggests that differential diagnosis of intraoral multiple lipomatous nodules should include not only lipomatosis, but also multiple classic lipomas, notwithstanding the rare incidence of the latter.     

颈部巨大良性对称性脂肪瘤病4例

良性对称性脂肪瘤病是发生在全身和颌面、头颈部或仅发生在颌面、头颈部的一种少见疾病。本文报道4例良性对称性脂肪瘤病。患者临床主要表现为颈部多发性、无痛性肿块及活动障碍,采用一次或分次彻底切除肿瘤组织,术后1例创口延期愈合,另3例患者采用分次切除肿瘤组织术后创口均一期愈合。术后随访1年余,4例患者的外形及颈部活动功能基本恢复正常,未见肿瘤复发。本文结合相关文献,对颈部良性对称性脂肪瘤病从病因、临床表现、诊断、治疗等进行了讨论。     

Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity. II. Myofibroma and myofibromatosis of the oral soft tissues

BACKGROUND: Myofibroma is a solitary benign tumor of myofibroblasts. Myofibromatosis describes multiple, simultaneous myofibromas at different sites in various organs. The clinico-pathologic correlations of myofibroma/myofibromatosis confined only to oral soft tissues were analyzed. METHODS: In the English language literature, 41 myofibroma and 12 myofibromatosis cases involving the oral soft tissues were found. From our files, three new myofibroma cases were added. RESULTS: Age at time of diagnosis of oral mucosa myofibroma ranged from birth to 70 years (mean 21.7 years), considerably higher than myofibroma in other parts of the body. Lesions occurred during the first decade (44%) and in the first year of life (17%). Male:female ratio was 1:1.6, contrary to the male predominance in other parts of the body. Common sites were the tongue (32%) and buccal mucosa (18%). Treatment was local excision, either complete (n = 13) or partial (n = 3), wide excision (n = 4), surgery, and chemotherapy (n = 1). Myofibromatosis involving oral soft tissues was diagnosed at birth in nine (75%) patients, within the first year in two, and as a young adult in one. Male:female ratio was 2:1. The tongue was the most common site (50%). Half the patients died of disseminated disease within a few days from birth, three were cured by partial or complete excision, and three experienced spontaneous regression. Histologically, oral mucosa myofibroma/myofibromatosis appearance agreed with findings in the literature. CONCLUSIONS: Myofibroma should be included in the clinical differential diagnosis of masses of the oral soft tissues, especially in the tongue and buccal mucosa of children and adolescents. Histological differential diagnosis includes benign and malignant spindle-shaped lesions. Treatment of choice is local excision.     

Bilateral multiple spindle cell lipomas of the tongue

Spindle cell lipoma (SCL) typically occurs in elderly men as a solitary lesion in the posterior neck and back, but less commonly also involves the oral cavity. Here, we describe a rare case of bilateral multiple SCLs of the tongue. The patient was a 72-year-old Japanese man with multiple painless soft nodules in the bilateral margins of the tongue. The patient was not obese, and had used alcohol moderately for more than 40 years. A clinical diagnosis of multiple tongue lipomas was made. The tumors were resected surgically, and they exhibited the histopathological features of SCL, composed of mature fat cells, collagen-forming CD34-positive spindle cells, and sparse mast cells. This suggests that differential diagnosis of intraoral multiple lipomatous nodules should include not only lipomatosis but also multiple SCLs, notwithstanding the rare incidence of the latter.     

Congenital infiltrating lipomatosis of the face: report of cases and review of the literature

Three cases of infiltrating congenital lipomatosis of the face are presented. These lesions shared the following morphologic criteria: 1) nonencapsulated congenital fatty tumor; 2) infiltration of adjacent muscle and soft tissue; 3) absence of lipoblast and malignant characteristics; 4) presence of fibrous elements with numerous nerve bundles and vessels; and 5) hypertrophy of subjacent bone. Although these tumors are benign, all three recurred after surgical excision. Extensive specimen sampling is advisable to exclude infiltrating lipoma, diffuse angiomatosis, infiltrating angiolipoma, and well-differentiated liposarcoma. Relationship with lipoblastomatosis and histogenesis of infiltrating congenital lipomatosis of the face are discussed.     

A large dermoid cyst of the neck. Case report

An elderly patient presented with a large asymptomatic submental swelling which had been present for about fifteen years. The clinical diagnosis was a dermoid cyst and the lesion was removed in toto. Histological examination confirmed the clinical diagnosis. Unless such lesions are very large and cause displacement of the tongue, there is usually no associated dyspnoea, dysphagia or dysphonia.     

Myoepithelioma of the tongue

Soft tissue myoepitheliomas, in contrast to salivary gland myoepitheliomas, are benign neoplasms that typically occur in the soft tissues of the extremities. Both are characterized by a multilobular proliferation of polygonal to fusiform cells embedded in a variably myxoid to chondromyxoid matrix. A histologically similar lesion that has a marked predilection for the anterior dorsum of the tongue has been referred to as ectomesenchymal chondromyxoid tumor. The morphological and immunohistochemical resemblance of soft tissue myoepitheliomas to ectomesenchymal chondromyxoid tumors has led to the use of these designations interchangeably. We present a case of myoepithelioma of the tongue and review the literature, with emphasis on the differential diagnosis and histogenesis of this lesion and pertinent nosologic considerations.     

Giant nevi: a review

Congenital nevi are benign neoplasms that are present at birth and composed of nevomelanocytes. Approximately 1-3% of all newborns have congenital pigmented nevi, and the number of nevi increases with age, peaking by late adolescence to 20-40 nevi in an individual. Giant congenital nevi are often defined as nevi that are greater than 20 cm in diameter in an adult, or nevi that occupy 2% or more of the body surface area. Histologically, nevi are transformed melanocytes, which are normally highly dendritic cells interspersed among basal keratinocytes. The genetic basis of these lesions is not known. Findings of a culture of melanocytes from such a lesion from a showed chromosome rearrangements involving 1p,12q, and 19p. The giant nevi might be associated to several diseases: neurocutaneous melanosis, diffuse lipomatosis, structural brain malformations, hypertrophy of skull bones, limb atrophy, skeletal asymmetry involving both soft tissue hyper-and hypoplasia, von Recklinghausen's disease and vitiligo. The risk of malignant change in giant nevi is probably the most contentious issue in its management. The consensus is that lesions are pre-malignant, but the purported incidence of malignancy varies wildly from 0-42%. Surgical excision remains the mainstay of treatment for large congenital melanocytic nevi, and most giant nevi are managed by staged excision and resurfacing with skin grafts or tissue expanders and flaps.     

Unusual presentation of nodular fasciitis as a rapidly growing haemorrhagic temporal mass

Nodular fasciitis (NF), also called proliferative or infiltrative fasciitis is considered to be a benign reactive process of the soft tissues (subcutaneous tissue, muscle or fascia) rather than a true neoplasm. Although the extremities are the most common sites, it may arise anywhere in the body. Local excision presents the main treatment. This study presents a case of NF with a unique clinical presentation, an acute subcutaneous temporal hemorrhagic growth. Because temporal NF is more often dermally situated than NF involving other anatomic sites, it may present with superficial ulceration or bleeding and appear clinically unusual. Nodular fasciitis should be considered in the differential diagnosis of other benign or malignant tissue masses involving the temporal area.     

Morbus Castleman

Case report

A 25-year-old patient with fever, dysphagia, and reduced general condition was referred to our department by his dentist after 1 week of antibiotic therapy. He presented with a painful palpable mass in the right lower jaw that had developed 2 weeks before. The orthopantomogram showed a caries and periapical lesion at the right lower wisdom tooth. During extraction of the tooth and putative intra- and extraoral abscess incision, no pus could be drained and the mass persisted. CT scans then revealed lobulated soft tissue masses on both sides of the neck with the main focus on the right side. In an additional operation with excision of the mass, Castleman’s disease of the hyaline vascular type was diagnosed.

Histopathologic findings

Staging could not detect further pathological findings and the patient’s postoperative general condition improved continuously. The histological features of the hayline vascular type of Castleman’s disease were characterized by multiple germinal centers surrounded by circumferentially arranged layers of small lymphocytes interconnected by a prominent vascular stroma with occasional plasma cells (“onion skin”).

Conclusion

Castleman’s disease is a rare and yet poorly understood disease, characterized by inhomogeneous growth of lymphoid tissue. Mostly benign it remains a diagnostic challenge before histological investigation. In unclear submandibular swellings and neck lumps assumed to be an abscess, this rare differential diagnosis must be considered. Facing recurrence and potential for malignancy, follow-up of the patients over several years is necessary.     

Severe symmetrical facial lipoatrophy in a patient with discoid lupus erythematosus

ABSTRACT: Acquired partial lipodystrophy is a rare disorder, and it is characterized by the absence of subcutaneous fat from the face, the neck, the trunk, and the upper extremities. The etiology of acquired partial lipodystrophy includes scleroderma and discoid lupus erythematosus. Literature review reveals studies involving 10 patients until today with lipoatrophy due to or after the onset of discoid lupus erythematosus; all are female patients. We want to report a young male patient with progressive symmetrical facial lipoatrophy. In addition, he has discoid lupus erythematosus and celiac disease. Fat grafting and adjuvant oral coenzyme Q10 tablets (Deka-none; Deka Pharmaceuticals, ?stanbul, Turkey) were administered for treatment. To our knowledge, this case involves the first male patient in the literature presenting with symmetrical facial lipoatrophy with very prominent periorbital lipoatrophy and bitemporal hollowing symptoms.     

Oral myoepithelioma of soft tissue origin: report of a new case and literature review

Oral myoepithelioma of soft tissue origin, an entity distinct from myoepithelioma of salivary glands, constitutes an extremely rare benign neoplasm, with only 1 previously published case, affecting the tongue of a 22-year-old woman. Ectomesenchymal chondromyxoid tumour (ECT) also is a very rare benign neoplasm of the oral cavity that has a strong predilection for the anterior dorsum of the tongue. The great similarities in the histologic and immunohistochemical characteristics of soft tissue myoepithelioma (STM) and ECT probably indicate that they refer to the same or closely related pathologic entities. The aim of this paper is to report a new case of STM affecting the dorsal tongue of a 45-year-old man. A review of the relevant literature is made and the relationship between oral STM and ECT discussed.     

Tongue atrophy in mixed connective tissue disease

A case is reported of tongue atrophy in a patient with mixed connective tissue disease (MCTD) and major myositic involvement. The case highlights oropharyngeal aspects of MCTD, including inability to wear dentures, dysarthria, and dysphagia. To our knowledge this is the first report of major tongue involvement in myositis as part of MCTD.     

口腔颌面部淀粉样变性病的临床病理研究

目的探讨全身系统性淀粉样变性病在口腔颌面部的临床病理特点。方法对１４例口腔颌面部淀粉样变性病的活检切片ＨＥ及刚果红等特殊染色、免疫组化、光镜和偏光显微镜超微结构观察。结果１４例的活体组织检查发现肿物为软组织和血管壁有粉红均质的淀粉样蛋白沉积，刚果红等特殊染色阳性，结合免疫组织化学染色可鉴别原发和继发性病，透射电镜观察淀粉样蛋白呈毛毡样的细丝状物。结论淀粉样变性病在口腔主要损害舌，其次为颊唇粘膜及涎腺