拇指近节骨窦细胞组织增生伴巨淋巴细胞病一例报告

患者女,52岁,以右拇指疼痛4个月,加重1个月入院.患者4个月前无明显诱因出现右手拇指疼痛,当时未予重视,自行外敷膏药,疼痛无明显好转.近1个月,患者右手拇指逐渐肿胀,局部红肿,局部中或轻度发热,偶尔全身低热.查体:全身浅表淋巴结无肿大,右手拇指肿胀,以近节指骨为重,色泽瘀暗,触诊质软,肤温较高,指间关节及掌指关节活动稍受限,近节指骨局部疼痛,指端血运、感觉良好.     

Spinal extranodal Rosai-Dorfman disease: A case report and literature review

IntroductionSpinal extranodal Rosai-Dorfman disease (RDD) is extremely rare. In this paper, we reported successful management of spinal extranodal RDD and reviewed medical literature.Case presentationA 19-year-old male presented with progressive bilateral leg weakness and back pain for two months before admission. He denied weight loss, fever, night sweats, and lymph node enlargement. On examination, his muscle strength of both legs was grade I with hyperreflexia. Magnetic resonance imaging of the spine (MRI) showed a thoracic extradural mass at a level of T6-T9, which was a heterogeneous hyperintense on T2W, STIR, and isointense on T1W and enhanced contrast vividly. We resected the tumor totally and decompressed the spinal cord. Pathology revealed a histiocytic tumor. Immunohistochemical staining was S100 (+), CD68 (+), CD45 (+), and CD1a (−). Postoperatively, his muscle strength improved gradually to grade IV after four months. Postoperative MRI of the spine showed no residual tumor. No further adjuvant therapy was indicated.Clinical discussionSpinal extranodal RDD has no specific symptoms and pathognomonic imaging features. CT and MRI of the spine are still the essential tools for diagnosing RDD, but biopsy is often mandatory for definitive diagnosis. There have not been consensus guidelines for treating RDD of the spine because of its rarity. Surgical resection remained the mainstay of treatment (78.8%), with or without adjuvant therapies.ConclusionSurgery is the treatment of choice for most cases, while steroid therapy, radiotherapy, and chemotherapy should be adjuvant treatment and tailored individually.     

Rosai-Dorfman disease mimicking meningiomatosis: a case report

Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system manifestations, particularly in the absence of nodal disease with clinical and radiological findings suggestive of meningioma, are extremely rare. Histopathology and immunohistochemistry are essential for a positive diagnosis. We report a case of RDD in a patient presenting multiple meningeal nodules with a review of the literature and discussion of differential diagnosis.     

胸椎Rosai-Dorfman病合并截瘫一例报告

Rosai-Dorfman病是一种少见的、病因不明的组织细胞增生性疾病,主要发生于淋巴结内,又称窦组织细胞增生症伴巨淋巴结病(sinus histiocytosis with massive lymphadenopathy,SHML),发生于淋巴结外者少见,而单独发生于脊椎者极罕见.     

骨骼系统多发罗道病1例

患者,男,50岁,左大腿近端酸胀不适感1个月入院。患者自诉1个月前无明显诱因出现左大腿近端酸胀不适,行走后症状加重。查体:左大腿上部轻度深压痛,无局部红肿及下肢麻木感,Feber及Thomas征阴性。实验室检查:血常规、尿     

胸椎孤立性Rosai-Dorfman病1例报告

罗道病（Rosai-Dorfman disease,RDD）又称窦组织细胞增生伴巨大淋巴结病（sinus hisliocytosis with massive lymphadenopathy,SHML）,是一种罕见的良性组织细胞增生性疾病,1969年Rosai和Dorfman对其做了详细描述,并正式命名为Rosai-Dorfman病或SHML。至今病因仍不明确,典型表现为双侧颈部淋巴结无痛性肿大伴有发热,约43%的病例累及结外器官,最常见的结外部位是头颈部、皮肤、软组织、鼻腔和眼眶、骨骼、肾脏、唾液腺和中枢神经系统。孤立性结外骨骼病变极为少见,约占总发病的2%,而累及脊柱的病例罕见,容易误诊。我们收治1例孤立的胸椎原发RDD并出现了压缩性骨折,报道如下。     

Cutaneous Rosai-Dorfman disease of the forearm: case report

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare non-neoplastic pathologic condition that frequently pursues a prolonged clinical course marked by exacerbations and remissions. Cutaneous RDD is even less common than cases involving lymph nodes. We present the case of a patient with long-standing Crohn's disease who developed cutaneous RDD in the forearm.     

Isolated cerebellar involvement in Rosai-Dorfman disease: case report

OBJECTIVE AND IMPORTANCE: Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare but well-recognized disorder characterized by an unusual proliferation of histiocytic cells. Intracranial localization is a rare manifestation of RDD. Only three cases of localization in the posterior fossa have been reported in the literature. The present report describes the first case, to our knowledge, of cerebellar localization of RDD. CLINICAL PRESENTATION: A 67-year-old woman was admitted to our institution with a 5-month history of cerebellar ataxia. Her medical history was unremarkable. The patient was alert and cooperative. No cranial nerve deficits were evident; Romberg positivity to the left side was recorded. No cutaneous abnormalities, lymphadenopathy, or hepatosplenomegaly were revealed by physical examination. Routine hematological and biochemical studies were normal except for the erythrocyte sedimentation rate, which was elevated. Radiologically, the lesion appeared as a well-defined and avascular mass in the right cerebellar lobe. Meningioma was considered the most likely diagnosis. TECHNIQUE: The patient underwent a suboccipital craniotomy with complete excision of the lesion. Microscopic examination of the operative specimen revealed the presence of a mixed cellular population with predominant mature histiocytes. A peculiar feature was the presence of lymphocytes and monocytes within the cytoplasm of histiocytes (emperipolesis). Immunohistochemical study of the histiocytes revealed strong positivity for S-100, CD-68 antigen, and vimentin. CONCLUSION: Involvement of the central nervous system in RDD appears to have a benign prognosis, especially in the absence of nodal diseases. Surgery is essential for diagnosis, and, when total removal is achieved, the outcome is generally good without risk of recurrence.     

Rosai-Dorfman disease presenting with multiple intracranial and intraspinal masses: a case report

Rosai-Dorfman disease (RDD), a systemic histioproliferative disorder, was first described by Rosai and Dorfman in 1969. However, only 41 cases involving the central nervous system have been reported. The authors present a rare case of RDD with multiple intracranial and spinal lesions mimicking multiple meningioma. A 59-year-old woman was admitted to our hospital with a history of bilateral visual impairment. MRI demonstrated multiple central nervous system lesions in the suprasellar region, right temporal convexity, left frontal convexity, left cerebello-pontine angle and C5 level of the spinal cord. Preoperative neuro-imaging studies suggested multiple meningioma. Subtotal removal of the suprasellar lesion was performed, followed by gamma knife radiosurgery to the residual lesion and the other intracranial lesions. All intracranial lesions disappeared following gamma knife radiotherapy. The spinal lesion, however, was completely excised one year after the initial operation following the appearance of sensory disturbance. Both intracranial and spinal lesions were diagnosed as RDD on histological examination. No recurrences have been detected on MRI two years after the initial operation. Seeing the good results already achieved by surgical removal of RDD, this case suggests that the combination of surgery and gamma knife radiotherapy might improve further.     

Rosai-Dorfman病颅骨侵犯1例

患者,女,30岁。5年前发现右侧额部头皮有一黄豆大结节,质软,无压痛、头痛及头皮周围软组织触痛,无发热、头昏、恶心、呕吐,无视物模糊、肢体麻木。一直未予重视。3个月前无明显诱因下感肿块逐渐增大,约鸡蛋大小。体格检查:精神反应正常,问答切题。心肺听诊无异常。右侧额部可见一大小约4cm×3cm头皮肿块,基底宽,肿块边界尚清,活动度差,质韧,顶部偏软,顶部无压痛,周边轻压痛,局部头皮无红肿、破溃,毛发无脱落,无血管扩张,未闻及血管杂音,皮温无殊。耳前、颌下及颈部等全身浅表淋巴结未及肿大。实验室检查:血、尿、便常规及肝功能正常。影像学表现:头颅CT示右侧额部头皮下见一大小约3.2cm×4.3cm宽基底的软组织肿块影,边缘清楚,密度较均匀,CT值约27.8HU,局部颅骨见不规则孔状骨质破坏,边界较清楚,未见明显硬化缘,局部头皮下脂肪层受压变薄(图1a-1d)。MRI示右侧额骨局     

Extranodal Rosai-Dorfman disease involving the bone marrow: a case report

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a very rare disorder of unknown etiology. Although classical SHML usually presents as massive, painless, bilateral neck lymph node enlargement, approximately 25% to 40% of SHML cases described to date present in extranodal organs or tissues, such as skin, upper respiratory tract, and bone etc. However, bone marrow as a site of initial presentation by SHML has not been described since the disease was initially recognized in 1969. Here, we report the first case of bone marrow involvement by extranodal SHML, which occured in a 73 year-old man with a history of refractory idiopathic thrombocytopenia purpura. The bone marrow biopsy demonstrates the distinctive and characteristic morphologic and immunophenotypic features of extranodal SHML.     

Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery. Case report

Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare idiopathic histioproliferative disorder typically characterized by painless cervical lymphadenopathy, fever, and weight loss. Extranodal, intracranial disease is uncommon. In this report the authors describe the first case of intracranial RDD treated with stereotactic radiosurgery after resection. This 52-year-old man with known RDD presented with a 7-day course of fever, headache, diplopia, left facial paresthesias, and difficulty swallowing. No cranial nerve deficits were evident on examination, but right submandibular and inguinal node enlargements were noted. On neuroimaging, the patient was found to have a homogeneously contrast-enhancing petroclival lesion with extension into the left cavernous sinus. The patient underwent a combined left petrosal craniotomy and partial labyrinthectomy with duraplasty for biopsy sampling and partial microsurgical resection of the lesion. Microscopic examination of the biopsy specimen revealed the presence of a mixed cellular population with predominant mature histiocytes consistent with RDD. The residual tumor was treated with stereotactic radiosurgery 2 months after resection. On follow-up imaging the lesion had regressed significantly, with only slight dural enhancement remaining. Microsurgical resection for histological diagnosis, followed by stereotactic radiosurgery for residual tumor represents one treatment alternative in the management of intracranial RDD in which a complete resection carries the potential for excess morbidity.     

Spinal epidural Rosai-Dorfman disease preceding by relapsing uveitis: a case report with literature review

STUDY DESIGN: Case report. SETTING: Tertiary referral center hospital in Taiwan. OBJECTIVES: To report a case of spinal Rosai-Dorfman disease (RDD) presenting with paraparesis and also preceding by relapsing uveitis for 6 months. A thoracic laminectomy was performed to remove the solid mass. The pathological diagnosis reveals infiltrating histiocytes, emperipolesis and positivity for S-100. There is no recurrence 1 year later with MR imaging. CONCLUSIONS: The relapsing idiopathic uveitis may be a prodrome for this unusual disease, because RDD is associated closely to defective immunogical response. Early and accurate diagnosis of CNS RDD may reverse the neurologic deficits by early decompression.     

Growing fracture of the orbital roof. A case report

We report a case of growing fracture of the orbital roof in a 5-year-old child. The presenting sign was a pulsatile orbital mass. This child had a history of a minor head injury with orbital impact 2 years ago. Cerebral CT scan revealed a diastatic fracture of the right orbital roof. On MRI a leptomeningeal cyst extending in the orbital cavity was shown. Frontal craniotomy with direct repair of the dural and bone defects was performed. The outcome was excellent. In the literature the exact pathophysiology of the growing fractures is still debated but a dural laceration along the fracture line is noted in all the cases. They are mostly located in the cranial convexity, and rarely affect the skull base. Only 5 similar cases were found in the relevant literature. Growing fracture of the orbital roof should be suspected if ocular symptoms appears in childs who have sustained a head injury several months or years ago.