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.     

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.     

颅内脑膜黑色素细胞瘤的临床与病理

目的:探讨颅内脑膜黑色素细胞瘤的临床与病理特征。方法:分析4例病人的临床表现、影像学检查、病理学特性、治疗方法及预后等资料。结果:光镜检查可见瘤细胞内有大量黑色素颗粒。免疫组织化学检查S-100蛋白、抗黑色素瘤抗体呈强阳性,而细胞角蛋白、上皮膜抗原、胶质纤维酸性蛋白呈阴性。术后随访1～5年半未见肿瘤复发。结论:脑膜黑色素细胞瘤是起源于软脑膜黑色素细胞的一类良性肿瘤,颅内任何部位均可发生。手术全切肿瘤并辅以放疗是治疗的最佳选择。     

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.     

Intermediate grade meningeal melanocytoma of cervical spine

Background  Meningeal melanocytoma is a rare, benign melanotic tumor of the leptomeninges, which occurs anywhere in the cranial or spinal regions but most commonly in supratentorial and thoracic spine regions. The literature on this entity consists of case reports; therefore, there is no agreement on the most effective therapy of this tumor, although total excision seems to be the best therapeutic option. Case history  We report a 17-year-old girl with intermediate grade meningeal melanocytoma involving the C6 nerve root with spinal cord compression resulted in progressive tetraparesis. Clinical and radiological examinations suggested the possibility of an intradural extramedullary solid mass. The tumor was removed subtotally through cervical laminotomy followed by rapid improvement of most neurological deficits. This tumor was unusual because of its very hyperintense homogenous signal on T1-weighted images, invasion of the arachnoid membrane, and extension into the neural foramina. Black dots on the surface of the cord were thought to represent an organized blood clot until the frozen section suggested a melanocytic tumor. Discussion  We discuss the distinction of meningeal melanocytoma from other melanocytic tumors of the leptomeninges. Conclusion  Melanocytic tumors should be considered in the differential diagnosis when a hyperintense lesion of the leptomeninges is identified on T1-weighted images or a very dark mass similar to charcoal or organized hematoma is found in the surgical field. The best management is complete tumor resection, but radiotherapy is reserved in cases of subtotal resection and multiple lesions. Locally aggressive nature of tumor and possibility of recurrence warrant regular follow-up.     

Intracranial meningeal melanocytoma associated with nevus of Ota

We report a rare intracranial meningeal melanocytoma associated with the nevus of Ota. The patient was 36-year-old man with a 2-week history of headache and difficulty in opening his right eye. Physical examination showed a black nevus scattered on the right-hand side of the face, right eyelid ptosis and papilledema. CT scans and MRI showed a tumor in the region of the right cavernous sinus. The tumor was subtotally resected. Histological examination confirmed the diagnosis of melanocytoma.     

Paediatric meningeal tumours

We review our experience of meningeal tumours in patients under 16 years who were treated between 1957 and 1993. These were 16 with meningiomas (median age 12 years) and 5 with poorly differentiated malignant meningeal tumours (median age 3 years). The incidence of meningeal tumours in this period was 1.62% of all CNS tumours. There were 19 supratentorial tumours (5 intraventricular), 1 tentorial and 1 cervical. Surgical excision was complete in 12 patients and incomplete in 6; in 2 patients a biopsy only was undertaken. The remaining patient had radiological diagnosis only. All 5 patients in the group with malignant poorly differentiated tumours died (median survival 5 months), confirming the aggressive nature of these lesions. Of the 16 patients with meningiomas, 4 died during follow-up but 11 of the 12 meningioma patients remaining alive had complete resections. There is a strong tendency for paediatric meningeal tumours to have a supratentorial origin and intraventricular location. The female preponderance found in adults is not evident in children. Incomplete surgical excision or focal neurological deficit at presentation was associated with poor outcome.     

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.     

Intrathecal cytostatic therapy of meningeal carcinomatosis

Summary Autoradiographic investigations with ³H-thymidine were performed on cerebrospinal fluid (CSF) cells from a case of meningeal carcinomatosis following carcinoma of the breast. The cells were found to be anaplastic histologically.Within a period of 12 days 3×25 mg methotrexate were injected into the subarachnoid space by lumbar (2×) or cisternal (1×) puncture. The CSF cells were reduced from 283/3 to 19/3, while the proportion of tumour cells fell from 90 to 2%. The labelling index before onset of therapy was 33%; it increased to 70% after the first intrathecal administration of cytostatic and finally fell to 23%. The mitotic index, which was generally less reliable, behaved in a parallel way; the initial value of 1.5% increased to 3% and then declined to values less than 0.5%. Despite detailed histological investigation, carcinomatous cells could not be found anywhere on the surfaces of the central nervous system or meninges.Clinically, the patient had never shown significant neuropathological or psychopathological findings. However, the headache which had been very severe during the meningeal carcinomatosis vanished completely after the second application of methotrexate.

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Zusammenfassung Autoradiographische Untersuchungen mit ³H-Thymidin wurden an Liquorzellen bei Meningealcarcinose nach Mammacarcinom vorgenommen. Der Tumor war histologisch weitgehend entdifferenziert. Unter intrathekaler Applikation von 3×25 mg Methotrexat^® innerhalb von 12 Tagen sanken die Zellzahlen von 283/3 auf 19/3 bei gleichzeitigem Rückgang des relativen Tumorzellanteils von 90 auf 2%. Der Markierungsindex lag vor Behandlungsbeginn bei 33%, stieg 4 Tage nach intrathekaler Cytostatica-Applikation auf 70% und sank auf 23% ab. Parallel verhielt sich der weniger verläßliche Mitoseindex, der von 1,5 über 3 auf Werte unter 0,5% fiel. Autoptisch wurden trotz eingehender histologischer Untersuchung der Oberflächen von ZNS und Meningen keine blastomatösen Infiltrate mehr festgestellt.Klinisch waren nie signifikante neuro- oder psychopathologische Befunde erhoben worden. Die anfangs ausgeprägten Kopfschmerzen waren nach der zweiten Instillation gänzlich geschwunden.

     

Meningeal carcinomatosis causing isolated bilateral symmetric progressive hearing loss

Abstract A 53-year-old man presented with isolated bilateral progressive hearing loss from about 5 months. Fourteen months after onset of this isolated symptom a diagnosis of meningeal carcinomatosis (MC) was made. Isolated bilateral deafness is a rare first manifestation of MC.     

Cerebral anaplastic pleomorphic xanthoastrocytoma with meningeal dissemination at first presentation

Introduction Pleomorphic xanthoastrocytoma (PXA) is a rare brain glial tumour found in young patients. Most cases are reported as evolving low-grade neoplasms associated with a long survival after complete surgical resection. Some PXAs, however, can demonstrate secondary malignant transformation or progress with a short survival. Anaplastic histological features at first presentation or secondary meningeal dissemination have rarely been reported.Case report We describe the case of a cerebral PXA in a 7-year-old girl presenting with anaplastic histological features and craniospinal meningeal dissemination that progressed rapidly with a poor outcome.     

Malignant melanoma in early Parkinson's disease: the DATATOP trial.

The available epidemiological data on the incidence of malignant melanoma in Parkinson's disease are contradictory. The role of levodopa therapy in this context has been debated. We identified all known cases of malignant melanoma (N = 5) in the DATATOP clinical trial cohort and compared that to published expected values (N = 1.5) for a standard healthy population. The standardized event ratio was 3.3 (95% confidence interval, 1.1-7.8), indicating that incidence of malignant melanoma was higher than expected. We found no association between levodopa therapy and the incidence of melanoma.     

Primary meningeal sarcoma with leiomyoblastic differentiation complicating pregnancy

Intracranial malignant tumors during pregnancy are rare. Primary meningeal sarcoma with leiomyoblastic differentiation during pregnancy has not been reported. We present the case of a 25-year-old woman in the third trimester of pregnancy with a large intracranial tumor destructing the parietal calvaria and invasion of soft tissues. Histological examination revealed primary meningeal sarcoma with leiomyoblastic differentiation. A gross macroscopical resection of the tumor with removal of the infiltrated parietal calvaria was performed after delivery of a healthy baby through caesarean section. A mass on the head rapidly enlarging during pregnancy should be considered for a malignancy of intracranial origin. Early radiological exams as well as tumor resection followed by staging and multimodality treatment should be urgently performed.     

Intraventricular Malignant Meningioma with CSF-Disseminated Spinal Metastasis : Case Report and Literature Review

The authors report a case of 42-year-old woman with an intraventricular tumor in the trigone of the left lateral ventricle. The first operation achieved a microscopically complete resection. The tumor was histologically atypical meningioma. After 26 months, there were recurrences of intraventricular meningioma. Complete resection of the tumor and adjuvant radiation therapy were performed, and the histological diagnosis was malignant meningioma. Sixteen months after the second operation, spinal metastasis in cervicolumbar lesion was diagnosed and a subtotal removal of cervical intradural extramedullary mass was performed. We describe an unusual case of intraventricular malignant meningioma with cerebrospinal fluid-disseminated spinal metastases with review of the clinical courses of previous reports.     

Spinal meningeal malformations in children (without meningoceles or meningomyeloceles)

Multiple meningeal malformations are described: anterior or lateral meningoceles, extradural meningeal cysts, and intradural arachnoid cysts. All diverticulae appear to be extensions of the subarachnoid space, producing symptoms early or later. It is impossible to unify all these lesions because they cause multiple pathological conditions, depending upon the anatomical form or level, other systemic malformations, spinal abnormalities, or associated familial diseases. Surgical treatment requires complete evaluation of each anatomical aspect before procedure.     

Personality and disease outcome in malignant melanoma

OBJECTIVE: The objective of this study is to evaluate the role of personality in the prediction of recurrence and survival times in early-stage malignant melanoma. METHODS: Sixty patients with Stage I malignant melanoma were assessed shortly after surgery and followed for 10-year outcome. Survival analyses were conducted (i.e., log-rank test and Cox proportional hazards regression method), with covariates and personality type as measured by the Eysenck Personality Questionnaire (EPQ). RESULTS: Men were three times more likely to recur and seven times more likely to die from malignant melanoma than were women. Every millimeter of tumor depth also increased the risks of both recurrence and death twofold. In contrast to medical indices, personality type could not be used to determine risk for either outcome. CONCLUSION: In the present study, personality type did not predict recurrence and survival times. Rather, malignant melanoma prognosis was overwhelmingly determined by disease biology.     

Clinical Implications of the Mitotic Index as a Predictive Factor for Malignant Transformation of Atypical Meningiomas

ObjectiveIntracranial atypical meningiomas have a poor prognosis and high rates of recurrence. Moreover, up to one-third of the recurrences undergo high-grade transformation into malignant meningiomas. We aimed to investigate the clinical factors that can predict the propensity of malignant transformation from atypical to anaplastic meningiomas. MethodsBetween 2001 and 2018, all patients with atypical meningioma, in whom the tumors had undergone malignant transformation to anaplastic meningioma, were included. The patients’ medical records documenting the diagnosis of atypical meningioma prior to malignant transformation were reviewed to identify the predictors of transformation. The control group comprised 56 patients with atypical meningiomas who were first diagnosed between January 2017 and December 2018 and had no malignant transformation. ResultsNine patients in whom the atypical meningiomas underwent malignant transformation were included. The median time interval from diagnosis of atypical meningioma to malignant transformation was 19 months (range, 7–78). The study group showed a significant difference in heterogeneous enhancement (77.8% vs. 33.9%), bone invasion (55.6% vs. 12.5%), mitotic index (MI; 14.8±4.9 vs. 3.5±3.9), and Ki-67 index (20.7±13.9 vs. 9.5±7.1) compared with the control group. In multivariate analysis, increased MI (odds ratio, 1.436; 95% confidence interval, 1.127–1.900; p=0.004) was the only significant factor for predicting malignant transformation. ConclusionAn increased MI within atypical meningiomas might be used as a predictor of malignant transformation. Tumors at high risk for malignant transformation might require more attentive surveillance and management than other atypical meningiomas.     

恶性脑膜瘤临床,影像与病理特征

目的 探讨恶性脑膜瘤的临床影像与病理特征。方法 复习１９７９￣１９９９年经治的１０例恶性脑膜瘤,应临床表现、影像、肿瘤类型病理检查、复发、放射治疗及随访进行研究。结果 全组病例均属恶性脑膜瘤,除一例存活外均已死亡。结论 ＣＴ对诊断恶性脑膜瘤有其临床意义。如肿瘤组织学表现有坏死、脑浸润、间变、有丝分裂增多、颅外转移、临床过程短暂或短期复发应归属恶性,特别是血管外皮细胞脑膜瘤应属高危性。     

Malignant meningioma as a second malignancy after therapy for acute lymphatic leukemia without cranial radiation

Rationale Meningiomas in the pediatric age group are very rare tumors, comprising about 1–4.2% of all primary pediatric intracranial tumors.Case report We present a 17-year-old patient who suffered from an intraventricular malignant meningioma. At the age of 2 years, acute lymphatic leukemia (common ALL [cALL]) was diagnosed and successfully treated with chemotherapy. There was no cranial radiation therapy.In December 2001, 13 years after diagnosis of cALL, he complained of headache, vomiting, and walking difficulties. Magnetic resonance imaging showed an enhancing mass with cystic components in the trigone of the right lateral ventricle. The tumor was removed completely. Histological diagnosis revealed a malignant papillary meningioma. After removal of a recurrent meningioma 16 months later, he received local radiotherapy.Conclusion Pathogenetic mechanisms, treatment options, and prognosis of meningiomas and secondary malignancies of this age group are discussed.     

脑膜癌病的临床与病理特点

目的探讨脑膜癌病的临床与病理特点。方法回顾性分析142例脑膜癌病患者的临床及脑脊液(CSF)细胞学资料和免疫细胞化学标记结果。结果本组患者临床特点:头痛123例(86.62%),头晕19例(13.38%),发热26例(18.31%),视物模糊10例(7.04%)。CSF检查及免疫标记:全部患者CSF中均发现癌细胞;癌细胞上皮膜抗原(EMA)( ),细胞角蛋白(CK)( ),CD14(-),胶质纤维酸性蛋白(GFAP)(-)。其中99例(69.72%)CK低分子( )、高分子(-),诊断为转移性腺癌;33例(23.24%)CK高分子( )、CK低分子(-),诊断为转移性鳞癌;10例(7.04%)CK高分子( )、CK低分子( ),考虑为腺鳞癌。105例找到原发灶,其中67例(47.18%)确诊为肺癌;23例(16.20%)在消化道内找到原发灶;9例(6.34%)为乳腺癌;子宫内膜癌、卵巢癌、肝癌各1例。另有37例(26.06%)未找到原发灶。诊断明确的患者中,15例失访,另90例中有82例(91.11%)于确诊后3～14个月死亡。未找到原发灶的患者中,有6例失访,另31例中有28例(90.32%)在确诊后2～13个月内死亡。结论脑膜癌病患者以头痛、头晕为主要临床表现,并可伴有发热及视物模糊。CSF细胞学检查是诊断脑膜癌病的主要依据,通过免疫细胞化学的方法,可以确定转移癌细胞。脑膜癌病患者预后较差。