Primary Malignant Melanoma in the Pineal Region

A 59-year-old male patient had 5-month history of gait disturbance and memory impairment. His initial brain computed tomography scan showed 3.5×2.8 cm sized mass with high density in the pineal region. The tumor was hypointense on T2 weighted magnetic resonance images and hyperintense on T1 weighted magnetic resonance images with heterogenous enhancement of central portion. The tumor was totally removed via the occipital transtentorial approach. Black mass was observed in the operation field, and after surgery, histopathological examination confirmed the diagnosis of malignant melanoma. Whole spine magnetic resonance images and whole body 18-fluoro-deoxyglucose positron emission tomography could not demonstrate the primary site of this melanoma. Scrupulous physical examination of his skin and mucosa was done and dark pigmented lesion on his left leg was found, but additional studies including magnetic resonance images and skin biopsy showed negative finding. As a result, final diagnosis of primary pineal malignant melanoma was made. He underwent treatment with the whole brain radiotherapy and extended local boost irradiation without chemotherapy. His preoperative symptoms were disappeared, and no other specific neurological deficits were founded. His follow-up image studies showed no recurrence or distant metastasis until 26 weeks after surgery. Primary pineal malignant melanomas are extremely rare intracranial tumors, and only 17 cases have been reported since 1899. The most recent case report showed favorable outcome by subtotal tumor resection followed by whole brain and extended local irradiation without chemotherapy. Our case is another result to prove that total tumor resection with radiotherapy can be the current optimal treatment for primary malignant melanoma in the pineal region.     

Cellular blue naevus of the scalp with brain invasion.

A case of a 28 year old woman with an intracranial cellular blue naevus (CBN) which was believed to be the extension from a pigmented skin/scalp lesion is reported. There was no similar pigmented skin lesion noted on other parts of her body. Radical intervention, including wide excision of the affected skin of the scalp, removal of the underlying pigmented skull bone and wide excision of the pigmented dura, together with wide excision of the intracranial mass, were performed. The skin defect was covered by rotation flap and free skin graft. The dura was closed by grafting with fascia lata. The skull defect was left open and would be repaired later at a second planned surgery.     

Malignant transformation of intracranial meningeal melanocytoma. Case report and review of the literature

Meningeal melanocytoma is an uncommon pigmented neoplasm that affects the CNS and develops in the cranial and spinal leptomeninges. Here we report on a case of malignant transformation of intracranial supratentorial meningeal melanocytoma which recurred after 3 years as malignant melanoma. This case demonstrates that the biological behavior of melanocytoma is uncertain and that these lesions may recur as malignant melanoma.     

Malignant meningeal melanoma

We report the case of an isolated pigmented lesion of the cerebellar tentorium which was initially interpreted as meningeal melanocytoma. The immunohistochemical study required as a result of a subsequent post-surgical recurrence prompted us to reconsider the early diagnosis in favour of malignant meningeal melanoma. On the basis of data in the literature, the possibility that the lesion may have been a dural melanoma is discussed.     

Malignant meningeal melanoma

We report the case of an isolated pigmented lesion of the cerebellar tentorium which was initially interpreted as meningeal melanocytoma. The immunohistochemical study required as a result of a subsequent post-surgical recurrence prompted us to reconsider the early diagnosis in favour of malignant meningeal melanoma. On the basis of data in the literature, the possibility that the lesion may have been a durai melanoma is discussed.     

Primary spinal melanoma with bilateral papilledema

A case of primary leptomeningeal malignant melanoma localized in the cervical region in a 41-year-old woman is presented. The only clinical finding was intracranial hypertension with papilledema. A diagnosis of primary CNS melanoma was made after dermatological and ophthalmological consultations, ruled out a metastatic lesion. Primary leptomeningeal melanoma is an extremely rare spinal tumor. Its clinical presentation with signs of increased intracranial pressure but without cord symptoms is unusual. Clinical features of this case including the radiological and histologic findings are described. Diagnosis as well as management is discussed.     

A case of cystic metastatic intracranial amelanotic melanoma--analysis of findings in CT and MRI

A case of cystic intracranial metastatic amelanotic melanoma is presented. As far as we know, cyst formation in intracranial melanoma is rare, and only 15 cases of intracranial amelanotic melanoma have been reported until now. A 63-year-old man was admitted with headache and progressive visual disturbance. CT scan revealed a large low-density mass with ring-like enhancement in the left frontal lobe. Both T 1-and T 2-weighted MRI images revealed hyperintensity. A left frontotemporal craniotomy was performed. A yellowish mass was observed in the frontal lobe. The content of the cyst consisted of old hematoma, xanthochromic fluid and necrotic tissue, was evacuated and the cyst wall was totally resected. No abnormal pigmentation was noted in the cyst wall and surrounding brain tissue. The histological examination revealed amelanotic melanoma. Primary lesion was found on the left thigh later and resected. The patient died of further intracranial metastasis with repeated hemorrhage 5 months after the admission. Both CT and MRI findings of our case is atypical as an intracranial malignant melanoma. However, these are compatible with those of intracerebral hemorrhage in subacute stage. It is suggested that melanoma may make the diagnosis difficult when tumor hemorrhage modifies the images of CT or MRI.     

颅内恶性黑色素瘤的诊断和治疗:15例报告

目的颅内黑色素瘤发病率较低,手术是治疗的主要方法,本文探讨颅内黑色素瘤的诊断与治疗与预后。方法回顾性分析15例临床病理诊断为颅内黑色素瘤的病例,所有患者均进行手术治疗,根据其影像学、病理与预后进行分析。结果颅内黑色素瘤的临床表现以占位效应、癫痫与局灶神经症状为主,CT与MRI表现多样,MRI典型表现只有5例,上皮样细胞是病理的主要类型共13例,溶解蛋白(S-100)的敏感性与人黑色素相关抗原(HMB-45)的特异性结合可以对诊断有所帮助。原发性颅内黑色素瘤患者平均生存期为12个月,继发性颅内黑色素瘤平均生存期为5个月。结论颅内黑色素瘤的临床表现缺乏特异性,手术大体所见与病理是确诊的主要手段,但患者总体预后较差,继发性颅内黑色素瘤较原发性颅内黑色素瘤更差。     

颅内转移性恶性黑色素瘤:一例报告并文献复习

目的探讨颅内转移性恶性黑色素瘤临床组织病理学及免疫组织化学特征。方法应用组织病理学及免疫组织化学检测方法对1例颅内多发转移性恶性黑色素瘤患者的两次手术标本进行观察,通过复习文献分析其组织病理学和免疫组织化学特征,提出诊断与鉴别诊断要点。结果男性患者,48岁。因颅内多发占位性病变、肿瘤卒中而行右侧额颞叶肿瘤切除术。两次手术标本组织病理学表现不一:首次右侧额叶病灶表现为异型性上皮样肿瘤细胞围绕毛细血管和纤维组织呈乳头状排列;再次手术标本为右侧颞叶,病灶表现为梭形细胞呈片巢状或束状排列,胞质丰富、淡染或透亮,胞核异型性明显、核仁呈明显嗜酸性。左侧腹股沟淋巴结活检提示肿瘤转移,且肿瘤组织局部可见大量黑色素颗粒。免疫组织化学染色肿瘤细胞弥漫表达S-100蛋白、人黑色素细胞瘤抗原45、Melan-A和波形蛋白,部分表达上皮膜抗原,而细胞角蛋白、高分子角蛋白、低分子角蛋白、胶质纤维酸性蛋白、突触素、神经微丝蛋白等表达阴性。结论颅内转移性恶性黑色素瘤的组织学表现复杂多样,临床病史和影像学资料对诊断具有重要参考价值,但明确诊断仍需依靠免疫组织化学检测结果。需注意与转移癌、脑膜肿瘤、淋巴瘤及其他含黑色素的颅内肿瘤相鉴别。     

原发性脊髓恶性黑色素瘤1例报告及文献复习

目的 探讨原发性脊髓恶性黑色素瘤的临床特点。方法 报告1例原发性脊髓恶性黑色素瘤的详细临床及病理资料，并结合献，对该罕见疾病的临床诊断、影像学、组织病理学及治疗等做一复习。结果 MRI显示肿瘤位于于髓外、硬脊膜下，以胸段为甚，波及全脊髓。T1加权稍高信号，T2加权呈高信号，增强后有明显强化。随着病程发展，肿瘤扩散至颅内。手术证实肿瘤位于软脊膜下并侵犯脊髓。组织病理、免疫组织化学电镜均表现为典型的恶性黑色素瘤特征。结论 该病例诊断为原发性脊髓恶性黑色素瘤，因肿瘤广泛扩散，术后化疗及免疫治疗效果不佳，发病约1年后死亡。     

Melanocytic meningitis and large congenital melanocytic naevus: neurocutaneous melanosis

Neurological symptoms in a patient with large congenital melanocytic naevus are highly suggestive of cerebromeningeal melanoma metastasis. The presence of melanocytic cells in cerebrospinal fluid confirms this diagnosis If their malignant nature is shared with cutaneous naevocytic cells. Conversely, neurocutaneous melanosis is diagnosed when benign melanocytosis meningitis is found in patients with multiple and/or large congenital melanocytic naevus, whether cutaneous naevus cells are benign or not, or when cerebrospinal fluid cells are malignant with benign cutaneous melanocytic naevus.We report the case of a young man aged 19 presenting with multiple and large congenital melanocytic naevus who experienced transcient neurological signs and increased intracranial pressure. Cerebral neuroimaging evoked meningeal infiltration which benign melanocytic nature was supposed on CSF analysis and confirmed by necropsy findings, only 3 month after neurological onset, leading to neurocutaneous melanosis diagnosis. This rare neuroectodermal dysembryoplasia finds expression in various neurological signs, depending on patient's age and leptomeningeal and/or cerebral proliferation localization. Lumbar puncture, cerebral scanography and MRI may help diagnosis, but only histological examination can prove neurocutaneous melanosis, more often by necropsy because of poor prognosis.     

Fulminant leptomeningeal carcinomatosis from a malignant melanoma arising in a cerebellopontine epidermoid cyst: A rare case with diagnostic pointers

A malignant component in an epidermoid cyst is rare. We report an exceptionally rare case of a malignant melanoma arising in an epidermoid cyst located in the cerebellopontine (CP) angle. A 26‐year‐old woman presented with headache, vomiting, ataxia and difficulty in swallowing over the previous 3 months. The radiological finding suggested an epidermoid cyst and the lesion was excised. The histopathology confirmed a CP angle epidermoid cyst. Within 1 month of discharge, she developed hydrocephalus for which a ventriculo‐peritoneal shunt was performed. Postoperatively she developed weakness in lower limbs. A contrast‐enhanced MRI was done which showed dilated CSF cisternal spaces with a small enhancing lesion in the pineal region and enhancement of meninges extending to the spinal cord. Re‐exploration showed gelatinous material with gross adhesions in the CP angle cistern. A dural biopsy was done which showed sheets of poorly differentiated tumor cells which expressed S100 and Melan A and were immunoreactive with Human Melanoma Black (HMB)‐45 antibody, consistent with the diagnosis of malignant melanoma. Histology of the excised epidermoid cyst was re‐evaluated in deeper sections and showed scattered atypical melanocytes in the basal layer of the epidermis which were highlighted with HMB‐45 antibody. The patient expired within 3 days of the second procedure due to respiratory failure. A very aggressive fulminant course of the disease was evident after surgery for the epidermoid cyst. Treatment options are limited. Criteria for identification of malignancy in an intracranial epidermoid cyst were identified in our case retrospectively and have been highlighted.     

Primary meningeal melanocytoma of the spinal cord: report of a paediatric case with benign course and review of the literature

A melanin producing encapsulated, intradural-extramedullar tumour in the thoracic spinal cord was surgically removed from a 12 year old girl. The rumour histologically proved to be a melanocytoma and the patient was treated with radiation therapy. During 18 years of follow up she has been free of symptoms. The clinical and pathological features of the case and the literature are reviewed. The importance of differentiating this benign lesion from meningeal malignant pigmented tumours is emphasized.     

Intracranial thrombosed anastomosing hemangioma: Case report

An anastomosing hemangioma is a relatively new diagnosis of a benign vascular lesion that is typically found in the genitourinary tract. On imaging, anastomosing hemangiomas have a broad differential diagnosis and can resemble malignant lesions such as angiosarcoma. Here we present a case of a 33-year-old male with seizures who on imaging was found to have a presumed recurrent intracranial meningioma. After surgical resection of his lesion, this case was pathologically diagnosed as having anastomosing hemangioma. To our knowledge, this is the first report of a case of a thrombosed anastomosing hemangioma located at intracranial and intradural region.     

Endoscopic transnasal resection of ameloblastoma with intracranial extension

Ameloblastoma is a rare odontogenic tumor with characteristics of epithelial tissue that produces enamel for the developing tooth. This lesion is generally considered benign, but has malignant forms that invade locally and metastasize. We present a 60-year-old man with maxillary ameloblastoma that after multiple recurrences developed intracranial extension with dural involvement of the middle cranial fossa and was treated by endoscopic transnasal resection followed by radiation therapy. Our technique and intraoperative findings are described with a review of the literature on intracranial ameloblastoma. This patient represents a unique account of endoscopic transnasal resection being utilized in the treatment of intracranial extension of ameloblastoma and demonstrates potential for application in similar cases.     

颅内转移性恶性黑色素瘤：一例报告并文献复习

目的探讨颅内转移性恶性黑色素瘤临床组织病理学及免疫组织化学特征。方法应用组织病理学及免疫组织化学检测方法对1例颅内多发转移性恶性黑色素瘤患者的两次手术标本进行观察,通过复习文献分析其组织病理学和免疫组织化学特征,提出诊断与鉴别诊断要点。结果男性患者,48岁。因颅内多发占位性病变、肿瘤卒中而行右侧额颞叶肿瘤切除术。两次手术标本组织病理学表现不一：首次右侧额叶病灶表现为异型性上皮样肿瘤细胞围绕毛细血管和纤维组织呈乳头状排列;再次手术标本为右侧颞叶,病灶表现为梭形细胞呈片巢状或束状排列,胞质丰富、淡染或透亮,胞核异型性明显、核仁呈明显嗜酸性。左侧腹股沟淋巴结活检提示肿瘤转移,且肿瘤组织局部可见大量黑色素颗粒。免疫组织化学染色肿瘤细胞弥漫表达S一100蛋白、人黑色素细胞瘤抗原45、Melan—A和波形蛋白,部分表达上皮膜抗原,而细胞角蛋白、高分子角蛋白、低分子角蛋白、胶质纤维酸性蛋白、突触素、神经微丝蛋白等表达阴性。结论颅内转移性恶性黑色素瘤的组织学表现复杂多样,临床病史和影像学资料对诊断具有重要参考价值,但明确诊断仍需依靠免疫组织化学检测结果。需注意与转移癌、脑膜肿瘤、淋巴瘤及其他含黑色素的颅内肿瘤相鉴别。     

An autopsy case of neurocutaneous melanosis associated with intracerebral malignant melanoma

The authors reported the clinical course and the postmortem examination of a unique case of neurocutaneous melanosis with numerous anomalies and complications, which included congenital dislocation of lenses, hypogonadism, ectopia of prostatic duct, genuine phimose, retentio testis, psina bifida and neurogenic bladder. This 13-year-old boy with a large hairy nevus in a bathing trunk configulation and multiple small nevi over the whole body since his birth was admitted to our hospital for evaluation of headache and vomiting. Neurological examination showed bilateral papilledema and slight left hemiparesis. A CT scan revealed a large right frontal mass and craniotomy was performed with subtotal removal of this tumor which was confirmed as a malignant leptomeningeal melanoma. He initially made uneventful postoperative recovery, and two courses of chemotherapy with DTIC, ACNU and VCR were given; however, the currence of brain tumor ensued shortly thereafter, and he died in approximately six months after the onset of intracranial symptoms despite of the third course of chemotherapy. Thirty five cases of neurocutaneous melanosis associated with or without malignant melanoma have been reported in Japan. Twenty-eight cases were male and 7 female. Two cases showed the evidence of primary malignant melanoma outside of the central nervous system, whereas twenty eight leptomeningeal melanoma, in which 22 were solid and 6 diffuse, were shown intracranially. Other 5 cases had epileptic seizure and/or hydrocephalus caused by wide spreaded leptmeningeal melanosis. This high incidence of intracranial malignant melanoma in this disorder was remarkable compaired with the previous reports in other countries. Mean duration between deaths and the onset of symptoms of intracranial hypertension or focal neurological signs was 7 months, ranging from 1 to 24 months, showing the rapidly deteriorating course in this disorder.(ABSTRACT TRUNCATED AT 250 WORDS)     

先天性黑色素细胞痣伴弥漫性黑色素细胞增多症恶性变1例并文献复习

目的探讨先天性黑色素细胞痣(CMN)伴弥漫性黑色素细胞增多症恶性变的治疗方法及效果。方法回顾性分析1例CMN伴弥漫性黑色素细胞增多症恶性变病人的临床资料,行显微手术切除病变组织,送病理检查。并对该病进行文献复习。结果病变达镜下全切除,病理检查结果符合恶性黑色素瘤(MM),综合临床特征,病理诊断考虑为弥漫性黑色素细胞增多症恶性变。病人术后未行进一步治疗,于术后3个月死亡。结论 CMN是一种先天性色斑样痣,仅极少部分可演变为MM和神经皮肤黑色素细胞增多症(NCM)并恶性变,大型CMN病人存在大量复合卫星痣是发生MM和NCM最重要的危险因素。临床上一旦出现颅内MM,治疗应以外科手术为主,放、化疗及免疫治疗仅可使极少部分病人受益,预后极差。     

Ganglioglioma occurring with glioblastoma multiforme: Separate lesions or the same lesion?

The authors report on the first such case of ganglioglioma and a malignant variant in the same individual without prior irradiation. Gangliogliomas are frequently encountered in children and young adults and have a predilection for the temporal lobes. Sporadic cases of malignant degeneration have been reported; however, most cases have undergone radiation or subtotal resection. A 45-year-old female was seen for speech abnormalities and symptoms referable to elevated intracranial pressure. The patient had no significant past medical history and no history of neurocutaneous disorders. Two separate lesions located in the posterior and anterior temporal lobes were found on imaging. At initial surgery, she underwent gross total resection of the anterior temporal tip ganglioglioma and cyst aspiration of the posterior temporal lobe lesion. The anterior temporal lesion was a ganglioglioma and did not recur. However, the posterior temporal lesion was identified as a malignant ganglioglioma/glioblastoma multiforme variant that recurred multiple times requiring several surgeries, radiation and chemotherapy. The occurrence of these distinct entities is uncommon in patients without a history of prior radiation treatment. Even rarer, is the occurrence of these separate intracranial lesions in a patient without a history of phacomatosis. For benign gangliogliomas, gross total resection can be curative; however, more aggressive variants may be resistant to multimodal therapies.     

The diagnostic dilemma of primary central nervous system melanoma

Melanomas are malignant neoplasms of melanocytes developing predominantly in the skin, but occasionally arising from eyes, mucous membranes, and the central nervous system (CNS). The CNS can be affected by a spectrum of melanocytic lesions ranging from diffuse neurocutaneous melanosis, to a focal and benign neoplasm (melanocytoma), and to an overtly malignant tumor (melanoma). Primary melanocytic lesions involving the CNS are typically concentrated in the perimedullary and high cervical region. Primary CNS melanoma cannot be reliably distinguished from metastatic melanoma on neuroimaging and histopathological characteristics alone: its diagnosis is established only after exclusion of secondary metastatic disease from a cutaneous, mucosal or retinal primary. We present two patients with primary CNS melanoma and discuss relevant issues, available treatment options, and expected outcomes. Awareness of disease spectrum and clinico-biological differences may be used to guide therapeutic decision-making for a patient with a proven or suspected primary CNS melanoma.