腰椎单骨型骨纤维结构不良1例及文献复习

目的:结合文献复习,探讨脊柱单骨型骨纤维结构不良的治疗方法及预后。方法:报告１例经影像学、病理证实腰椎骨纤维结构不良的患者,行前路腰3椎体病灶及附件切除、L2-4间钛网置入脊柱前柱重建、植骨融合、MACS内固定术,并回顾相关文献。结果:术中切除部分腰3椎体及附件,重建脊柱稳定,病理检查结果提示:（L3椎体肿瘤）纤维结构不良,免疫组化结果示:CAM5.2(-)、CKpan(-)、EMA(-)。术后病人疼痛症状缓解,恢复良好。结论:脊柱单骨型纤维结构不良比较罕见,经前路病灶清除、植骨内固定可获得良好的治疗效果。     

FIbrous dysplasia of right upper jaw — uncommon presentation

30 years, male patient presented with huge nasal mass for 25 years and nasal obstruction for 15 years. On examination, a mass of 10“ × 8 ” size found involving the right side of face, nose, cheek, orbit and palate. The right eye was proptosed eccentrically and was completely blind. Histopathological examination confirmed it as fibrous dysplasia.     

Fibrous dysplasia of right upper jawuncommon presentation

MR, 30 years, M/M had huge nasal mass for 25 years and nasal obstruction for 15 years. On examination, a huge mass involving right side of face, nose, cheek, orbit and palate of 10″ × 8″ size was noticed. The right eye was proptosed eccentrically and was completely blind. Histopathological examination diagnosed the case to be fibrous dysplasia.     

Fibrous dysplasia of the sphenoid sinus in an adolescent male.

Fibrous dysplasia involving the sphenoid sinus and the entire left sphenoid bone, manifested clinically by left proptosis and frontal headaches, was diagnosed in a 13-year-old black male. The case is unusual since it is only the third reported instance of fibrous dysplasia of the sphenoid sinus, and because the disease became clinically apparent at the onset of puberty in the patient. Since previous reporters have suggested that fibrous dysplasia remains stable or progresses slightly after the onset of puberty, the patient was not treated with radical surgery, but instead was observed closely. At this point, one year following admission, the patient's proptosis has disappeared and his symptoms are markedly improved.     

Fibrous dysplasia

Three instances of fibrous dysplasia are reported. In one, only the left maxilla was involved, in the second, the maxilla plus the ethmoid were involved, while in the third the right and left maxillae alongwith the ethmoid formed one unit of the lesion. The last named showed a very advanced state of the disease process resulting in destructive changes and cyst formation. None showed skin pigmentation or extraskeletal lesions. In view of these clinical presentations, the relevence of the prefixes “Monostotic or Polyostotic” to fibrous dysplasia has been discussed.     

Mazabraud’s syndrome: Intramuscular myxoma associated with fibrous dysplasia

The association of fibrous dysplasia and intramuscular myxoma is a rare disease known as Mazabraud’s syndrome. Both lesions tend to occur in the same anatomical region. The relationship between fibrous dysplasia and myxoma remains unclear, where an underlying localized error in tissue metabolism has been proposed to explain this occasional coexistence. Another example of this syndrome in a 52 year-old woman is reported. The patient presented with a soft tissue mass at the anteromedial mid part of the left thigh. After excision of the mass, three separate bone lesions were detected in her control MRI. The soft tissue mass was misdiagnosed as liposarcoma in another center, and the bone lesions were interpreted as metastasis. The hypocellularity and the indistinct vascular pattern of the lesion were consistent with myxoma. The Jam-Shidi needle biopsies of the osseous lesions were diagnosed as fibrous dysplasia. The recognition of this entity is important for appropriate management of the patient. Patients with soft tissue myxomas should be thoroughly examined for fibrous dysplasia. The greater risk of sarcomatous transformation in fibrous dysplasia with Mazabraud’s syndrome should also be kept in mind. (Pathology Oncology Research Vol 10, No 2, 121–123)     

Osteogenic Sarcoma Arising in Polyostotic Fibrous Dysplasia A Case Report

A 66 year old man with a longstanding history of polyostotic fibrous dysplasia was found to have an osteogenic sarcoma arising from the upper end of the left femur. As he was surgically unfit, he was treated with a course of external radiotherapy, with good palliation.     

Imaging of craniofacial fibrous dysplasia

Fibrous dysplasia is a relatively common disorder of bone. It may affect the bones of the face and skull and, in so doing, produce a wide variety of clinical presentations. Plain film assessment of craniofacial fibrous dysplasia may be difficult because of varying appearances and complex, overlapping structures. The MRI appearances of fibrous dysplasia are often non-specific and may be confusing. Findings on CT are also variable, but more commonly lead to a specific diagnosis. This is because of the characteristic ground-glass appearance of woven bone, seen on CT in most if not all cases of craniofacial fibrous dysplasia.     

Adenoid cystic carcinoma of nasal cavity - a case report

A 38 year old male patient presented with pain and swelling on left side of nose with mass in left nostril. The other symptoms were nasal obstruction, epistaxisc from left nostril and epiphora from left eye. CT scan showed a mass arising from lateral wall of nose on left side eroding anterior part of medial wall of maxillary sinus. The tumor was excised with wide margin through a lateral rhinotomy approach under general anaesthesia. In histopathologieal examination it was found to be cribriform type of Adenoid Cystic Carcinoma. This case is reported here for its rarit.     

Breast cancer in a patient with McCune-albright syndrome

We treated a patient with breast cancer associated with McCune-Albright syndrome. A 40-year-old woman with a history of precocious puberty visited our hospital complaining of a mass in the upper lateral quadrant of the right breast. Although bone scintigraphy revealed multiple high uptake of^99mTc, plain X-ray demonstrated ground-glass appearance, suggesting fibrous dysplasia rather than bone métastases. Serum levels of tumor markers and alkaline phosphatase were within the normal range. Breast cancer associated with McCune-Albright syndrome was diagnosed, and she subsequently underwent breast conserving surgery, excision of abdominal wall myxoma and bone biopsy of the left clavicula. The bone lesion was histologically confirmed as fibrous dysplasia. Although McCune-Albright syndrome is a rare clinical entity, it should be considered as a possible differential diagnosis of bone metastasis in patients with breast cancer. As recent molecular studies have suggested genetic mutations in McCune-Albright syndrome, this syndrome may possibly predispose patients to breast cancer.     

纤维结构不良恶性变一例报告并文献复习

目的：探讨纤维结构不良恶变的临床病理特征及其诊断和鉴别诊断。方法：分析了1例纤维结构不良骨肉瘤变患的临床表现，放射学及病理形态学的特征，并就病因及鉴别诊断等问题进行了讨论。结果：纤维结构不良发生恶变是一种罕见病变，其诊断必须依赖于临床、放射、病理三的结合。     

Fibrous dysplasia of Sphenoid bone

Fibrous dysplasia represents a disturbance of normal bone development specifically a defect in osteoblastic differentiation and maturation that originates in the mesenchymal precursor of the bone. In fibrous dysplasia, the lesion expands, which leads to a distortion and weakening of bone. Although the lesion is not encapsulated, it tends to remain enclosed within a shell of cortical bone. This shell can be thinned as a result of the pressure exerted on it. As it slowly progresses, fibrous dysplasia can cause skeletal destruction and deformity.     

Polyostotic Fibrous Dysplasia Mimicking Multiple Bone Metastases in a Patient with Ductal Carcinoma In Situ

Whole-body bone scans and whole body ¹⁸F-fluorodeoxyglucose positron emission tomographic/computed tomographic scans are sensitive for detecting bone metastasis in patients with breast cancer. However, it is often difficult to discriminate between bone metastasis and other nonmalignant bone lesions. Polyostotic fibrous dysplasia is a rare disorder characterized by the osteoid medullary cavity filling with fibrous tissue causing bony expansion. We report the case of a 42-year-old female patient with ductal carcinoma in situ, which appeared to have multiple bone metastases on initial work-up images. Subsequently, the bone metastases were identified as polyostotic fibrous dysplasia. The patient underwent modified radical mastectomy and subsequently visited for a second opinion regarding the bony metastases. She underwent right ilium computed tomography-guided biopsy. Pathology was consistent with fibrous dysplasia. This patient received only adjuvant tamoxifen, and 1.5 years later, there was no evidence of recurrence.     

Rare,Primary Iliac,Pubic and Ischial Tumours in Children (Report of 14 Cases) — Part II

14 cases of rare, primary iliac, pubic and ischial bone tumours or tumorous conditions are reported. These include aneurysmal bone cyst, eosinophilic granuloma, cavernous haemangioma, osteoid osteoma, fibrous dysplasia, fibrous dysplasia with sarcomatous degeneration, chondrosarcoma, lymphoma and atypical malignant histiocytosis. The possibilities to be considered in the accurate radiographic recognition of primary tumours of iliac, pelvic and ischial bones are discussed.     

A case of malignant fibrous histiocytoma after breast conserving therapy for breast cancer

A 45-year-old woman with malignant fibrous histiocytoma (MFH) of the breast following breast conserving therapy (BCT) is described. She noticed a lump in her left breast 52 months after BCT for breast cancer. The lump was excised and nodular fasciitis was initially diagnosed. However, the tumor recurred locally 4 times in the next 18 months. MFH was finally diagnosed. This case is considered to be radiation-induced sarcoma. The risk of radiation-induced sarcoma after BCT seems to be very low, however careful follow-up is necessary.     

Pleomorphic adenoma of nasal septum

The pleomorphic adenoma is the most common benign tumour of the major and minor salivary glands. We report a 40-year-old female patient, who presented with progressive swelling and deformity of the left side of her nose for last 2 years. On a lateral rhinotomy a mass (4 cm X3 cm in size) was found to be arising from the cartilaginous part of the nasal septum. The mass was emicleated from its capsule and the walls excised. The histopathological examination revealed it to be pleomorphic adenoma.     

Fibrous dysplasia of the skull with acromegaly and sarcomatous transformation

Summary Two cases of fibrous dysplasia of the skull are reported. Both patients were young women with acromegaly and were treated with radiotherapy. Progressive pareses of cranial nerves, pain, and a malignant course of the disease were characteristic in both patients, and the diagnosis of osteogenous sarcoma proved in one of them by histological examination.The clinical picture of fibrous dysplasia of the skull and the role of radiotherapy with the risk of development of malignancy is discussed.     

Huge pleomorphic adenoma of nasal cavity

Pleomorphic adenoma is a common benign tumour of the salivary gland that has elements of both epithelial and mesenchymal tissue. It is commonly seen in major salivary glands, parotid being the most common site followed by submandibular gland. However it can sometimes arise from minor salivary glands that are distributed throughout the upper aerodigestive tract and paranasal sinuses. We report a case of a 45 yrs. Old male patient, who presented with progressive nasal blockage and intermittent minor episodes of epistaxis from left nostril for 1 ½ years. Lateral Rhinotomy revealed a well encapsulated lesion attached to the nasal septum, which was excised completely. Histopathology revealed it to be Pleomorphic Adenoma without any features of malignancy. Lesion is a rare one hence reported.     

Intravascular papillary endothelial hyperplasia of the breast--report of a case with scanning electron microscopic observations

A 65-year-old woman noticed a left breast mass and underwent excisional biopsy. The excised mass measured 1.7 cm in diameter. Histologically, the tumor-like mass revealed irregular vascular channels with occasional thrombi and many papillary projections of fibrous stalks lined with a layer of flat endothelial cells. A small cavernous hemangioma was present adjacent to the lesion. Scanning electron microscopic observation revealed disorderly oriented thin and thick cords forming branches and meshworks in the lumina. The luminal wall and cords were covered with flat endothelial cells. Light and scanning electron microscopic differences between intravascular papillary endothelial hyperplasia and angiosarcoma are discussed.