AIM： To investigate the findings of infrared fundus autofluorescence（IR-AF） and spectral-domain optical coherence tomography（SD-OCT） in eyes with optic disc melanocytoma（ODM）.· METHODS： IR-AF findings and...AIM： To investigate the findings of infrared fundus autofluorescence（IR-AF） and spectral-domain optical coherence tomography（SD-OCT） in eyes with optic disc melanocytoma（ODM）.· METHODS： IR-AF findings and those of other ophthalmologic imaging examinations, including short-wave autofluorescence（SW-AF）, fluorescein angiography（FA）, fundus color photography, and SD-OCT of 8 eyes of 8 consecutive cases with ODM were assessed.·RESULTS： The ODMs in all cases（100%） presented similar IR-AF, SW-AF, and FA findings. On IR-AF images, ODMs showed outstanding hyper-AF with well-defined outline. On SW-AF images, the area of ODMs presented as hypo-AF. FA images revealed the leaking retinal telangiectasia on the surface of the ODMs. On SDOCT images in 8 cases（100%）, the ODMs were sloped with highly reflective surface, which were disorganized retina and optic nerve layers. In 7 cases（87.5%）, peripapillary choroids were involved. The melanocytomas of 8 cases（100%） presented as optically empty spaces. Vitreous seeds were found in one case（12.5%）.· CONCLUSION： IR-AF imaging may provide a new modality to evaluate the pathologic features of ODMs,and together with SW-AF imaging, offers a new tool to study biological characteristics associated with ODMs.SD-OCT is a valuable tool in delimitating the tumor extension and providing morphological information about the adjacent retinal tissue.展开更多
BACKGROUND Primary intramedullary melanocytoma is an exceedingly rare type of primary melanocytic tumor in the central nervous system.Unfortunately,primary intramedullary melanocytoma lacks specificity in clinical sym...BACKGROUND Primary intramedullary melanocytoma is an exceedingly rare type of primary melanocytic tumor in the central nervous system.Unfortunately,primary intramedullary melanocytoma lacks specificity in clinical symptoms and imaging features and there is currently no standard strategy for diagnosis or treatment.CASE SUMMARY A 52-year-old male patient suffered from weakness and numbness involving the bilateral lower limbs for 18 mo,and defecation and erectile dysfunction for 6 mo.Furthermore,these symptoms started to worsen for the last 3 mo.Preoperative magnetic resonance imaging(MRI)revealed an intramedullary tumor located at the T9-T10 level.In subsequently surgery,the maximal safe resection extent approached to 98%.The lesion was confirmed to be melanocytoma by pathological examination.In addition,the possibility of original melanocytoma outside the spinal cord was excluded after the examination of the whole body.Therefore,a diagnosis of primary intramedullary melanocytoma was established.The patient refused to accept radiotherapy or Gamma Knife,but MRI examination on July 28,2020 showed no sign of development.In addition,on April 10,2021,the recent review showed that the disorder of defecation and lower limbs improved further but erectile dysfunction benefited a little from the surgery.CONCLUSION After diagnosing intramedullary melanocytoma by postoperative pathology,the inspection of the whole body contributed to excluding the possibility of metastasis from other regions and further suggested a diagnosis of primary intramedullary melanocytoma.Complete resection,adjuvant radiation,and regular review are critical.In addition,maximal safe resection also benefits prognosis while the tumor is difficult to be resected totally.展开更多
Primary meningeal melanocytoma(MM)in the cerebellopontine angle(CPA)region is an extremely rare neoplasm that originates from the melanocytes in the leptomeninges.These lesions are usually misdiagnosed as they mimic o...Primary meningeal melanocytoma(MM)in the cerebellopontine angle(CPA)region is an extremely rare neoplasm that originates from the melanocytes in the leptomeninges.These lesions are usually misdiagnosed as they mimic other common CPA lesions through their nonspecific presenting symptoms,signs,and radiological characteristics.Here,we report a 47-year-old Chinese female patient who presented with a 1-month history of the right-sided tongue numbness and 1-week history of the right-sided face numbness that had been worsening for 2 days.The tumor,in the right CPA region,showed a slight isointensity on T1-weighted image and mixed signal intensity on T2-weighted image.The clinical presentation,surgical treatment,and pathologic characteristics were determined.The tumor was microsurgically resected and gross-total resection was achieved.The tumor revealed a solid,capsulated,brown-black lesion.Immunohistochemistry showed that the tumor cells were positive for human melanoma black-45(HMB-45),melanoma antigen(MelanA),S100,SOX10,and BRAF,confirming the final diagnosis of meningeal melanocytoma.Ultimately,no signs of radiological local recurrence were observed during the two-year follow-up.Collectively,meningeal melanocytoma is difficult to distinguish from common tumors in the CPA region before operation due to the lack of specificity in imaging and symptoms.Complete surgical resection is the best therapeutic option for this tumor.Although the tumor is commonly considered as a benign lesion,recurrence and metastasis are common,and pathogenesis remains unclear.展开更多
LIMAS and Tio1 proposed the term meningeal melanocytoma first time in 1972 to describe a primary melanotic tumor of the leptomeninges with prolonged clinical course and benign histology.Meningeal melanocytoma of the c...LIMAS and Tio1 proposed the term meningeal melanocytoma first time in 1972 to describe a primary melanotic tumor of the leptomeninges with prolonged clinical course and benign histology.Meningeal melanocytoma of the central nervous system is rare and benign primary meningeal melanocytoma (PMM) is more exceptional,and also less usual than the malignant types.2 This rare tumor falls under the subclassification of primary melanocytic lesions in the World Health Organization's classification of central nervous system tumors.3 PMM located in the foramen magnum region is an unusual cause of bulbus medullae and fourth ventricle compression.Here we report a 48-year-old man with a PMM which is located in the foramen magnum inducing supratentorial obstructive hydrocephalus.展开更多
Melanocytoma is a specific variant of melanocytic nevus, located in the optic disk or anywhere in the uveal tract, characterized clinically by a dark-brown to black color, and composed histopathologically of deeply pi...Melanocytoma is a specific variant of melanocytic nevus, located in the optic disk or anywhere in the uveal tract, characterized clinically by a dark-brown to black color, and composed histopathologically of deeply pigmented round to oval cells with small, round, uniform nuclei. In this report, we described a case of large juxtapapillary melanocytoma with atypical presentations.展开更多
目的：探讨中枢神经系统黑色素细胞瘤的临床病理特征。方法：对1例发生于脊髓的黑色素细胞瘤的临床表现、组织形态、免疫组织化学等进行分析,并复习相关文献。结果：患者女性,5 2岁,右侧肢体活动不灵活9个月。M R I示颈3、4椎体水平见...目的：探讨中枢神经系统黑色素细胞瘤的临床病理特征。方法：对1例发生于脊髓的黑色素细胞瘤的临床表现、组织形态、免疫组织化学等进行分析,并复习相关文献。结果：患者女性,5 2岁,右侧肢体活动不灵活9个月。M R I示颈3、4椎体水平见一软圆形界限清楚肿块,大小约3.7 cm×0.8 cm×1.3 cm。镜检示肿瘤细胞排列呈巢团状、漩涡状及束状;瘤细胞呈软圆形或梭形,部分细胞呈上皮样,大部分细胞质内见多量黑色素颗粒;瘤细胞无明显异型,无核分裂像。免疫组织化学Vimentin、HMB45、S-100、Melan-A均为阳性,Ki67指数5%-10%;其余Ckpan、EMA、GFAP、CD34、SMA、Desmin和P53均为阴性。结论：黑色素细胞瘤是一种罕见的低度恶性中间型肿瘤,临床上易与弥漫性黑色素细胞增多症和黑色素瘤病、恶性黑色素瘤以及伴有黑色素分化的其他肿瘤混淆。其诊断主要靠病理形态学特征、免疫组化、影像学及电镜检查有助于诊断与鉴别诊断。治疗主要依靠肿块切除,必要时可局部放疗,术后随访非常重要。展开更多
文摘AIM： To investigate the findings of infrared fundus autofluorescence（IR-AF） and spectral-domain optical coherence tomography（SD-OCT） in eyes with optic disc melanocytoma（ODM）.· METHODS： IR-AF findings and those of other ophthalmologic imaging examinations, including short-wave autofluorescence（SW-AF）, fluorescein angiography（FA）, fundus color photography, and SD-OCT of 8 eyes of 8 consecutive cases with ODM were assessed.·RESULTS： The ODMs in all cases（100%） presented similar IR-AF, SW-AF, and FA findings. On IR-AF images, ODMs showed outstanding hyper-AF with well-defined outline. On SW-AF images, the area of ODMs presented as hypo-AF. FA images revealed the leaking retinal telangiectasia on the surface of the ODMs. On SDOCT images in 8 cases（100%）, the ODMs were sloped with highly reflective surface, which were disorganized retina and optic nerve layers. In 7 cases（87.5%）, peripapillary choroids were involved. The melanocytomas of 8 cases（100%） presented as optically empty spaces. Vitreous seeds were found in one case（12.5%）.· CONCLUSION： IR-AF imaging may provide a new modality to evaluate the pathologic features of ODMs,and together with SW-AF imaging, offers a new tool to study biological characteristics associated with ODMs.SD-OCT is a valuable tool in delimitating the tumor extension and providing morphological information about the adjacent retinal tissue.
基金Natural Science Foundation of Hunan Province,China,No.2019JJ50964.
文摘BACKGROUND Primary intramedullary melanocytoma is an exceedingly rare type of primary melanocytic tumor in the central nervous system.Unfortunately,primary intramedullary melanocytoma lacks specificity in clinical symptoms and imaging features and there is currently no standard strategy for diagnosis or treatment.CASE SUMMARY A 52-year-old male patient suffered from weakness and numbness involving the bilateral lower limbs for 18 mo,and defecation and erectile dysfunction for 6 mo.Furthermore,these symptoms started to worsen for the last 3 mo.Preoperative magnetic resonance imaging(MRI)revealed an intramedullary tumor located at the T9-T10 level.In subsequently surgery,the maximal safe resection extent approached to 98%.The lesion was confirmed to be melanocytoma by pathological examination.In addition,the possibility of original melanocytoma outside the spinal cord was excluded after the examination of the whole body.Therefore,a diagnosis of primary intramedullary melanocytoma was established.The patient refused to accept radiotherapy or Gamma Knife,but MRI examination on July 28,2020 showed no sign of development.In addition,on April 10,2021,the recent review showed that the disorder of defecation and lower limbs improved further but erectile dysfunction benefited a little from the surgery.CONCLUSION After diagnosing intramedullary melanocytoma by postoperative pathology,the inspection of the whole body contributed to excluding the possibility of metastasis from other regions and further suggested a diagnosis of primary intramedullary melanocytoma.Complete resection,adjuvant radiation,and regular review are critical.In addition,maximal safe resection also benefits prognosis while the tumor is difficult to be resected totally.
文摘Primary meningeal melanocytoma(MM)in the cerebellopontine angle(CPA)region is an extremely rare neoplasm that originates from the melanocytes in the leptomeninges.These lesions are usually misdiagnosed as they mimic other common CPA lesions through their nonspecific presenting symptoms,signs,and radiological characteristics.Here,we report a 47-year-old Chinese female patient who presented with a 1-month history of the right-sided tongue numbness and 1-week history of the right-sided face numbness that had been worsening for 2 days.The tumor,in the right CPA region,showed a slight isointensity on T1-weighted image and mixed signal intensity on T2-weighted image.The clinical presentation,surgical treatment,and pathologic characteristics were determined.The tumor was microsurgically resected and gross-total resection was achieved.The tumor revealed a solid,capsulated,brown-black lesion.Immunohistochemistry showed that the tumor cells were positive for human melanoma black-45(HMB-45),melanoma antigen(MelanA),S100,SOX10,and BRAF,confirming the final diagnosis of meningeal melanocytoma.Ultimately,no signs of radiological local recurrence were observed during the two-year follow-up.Collectively,meningeal melanocytoma is difficult to distinguish from common tumors in the CPA region before operation due to the lack of specificity in imaging and symptoms.Complete surgical resection is the best therapeutic option for this tumor.Although the tumor is commonly considered as a benign lesion,recurrence and metastasis are common,and pathogenesis remains unclear.
文摘LIMAS and Tio1 proposed the term meningeal melanocytoma first time in 1972 to describe a primary melanotic tumor of the leptomeninges with prolonged clinical course and benign histology.Meningeal melanocytoma of the central nervous system is rare and benign primary meningeal melanocytoma (PMM) is more exceptional,and also less usual than the malignant types.2 This rare tumor falls under the subclassification of primary melanocytic lesions in the World Health Organization's classification of central nervous system tumors.3 PMM located in the foramen magnum region is an unusual cause of bulbus medullae and fourth ventricle compression.Here we report a 48-year-old man with a PMM which is located in the foramen magnum inducing supratentorial obstructive hydrocephalus.
文摘Melanocytoma is a specific variant of melanocytic nevus, located in the optic disk or anywhere in the uveal tract, characterized clinically by a dark-brown to black color, and composed histopathologically of deeply pigmented round to oval cells with small, round, uniform nuclei. In this report, we described a case of large juxtapapillary melanocytoma with atypical presentations.
