Intracranial granulocytic sarcoma in a patient with acute myeloid leukemia]

Granulocytic sarcoma (GS) is extramedullary tumor composed of immature leukemic cells. GS is presenting usually as a complication during the course of hematologic neoplasm, such as acute myeloblastic leukemia as well as myeloproliferative and myelodysplastic syndrome. The tumor was also called chroloma based on the green color of the tumorous mass. Central nervous system manifestations of GS are extremely rare. We report a 41-year-old man with acute leukemia type M7, who developed GS in the right occipital lobe after complete remission was achieved. Operative findings revealed the color of the hard tumor was greenish, which suggested the tumor was chroloma. Histological findings showed the tumor was GS. The majority of reported cases of GS in acute myeloid leukemia were M2 FAB classification and have chromosome translocation. Our patient was M7 FAB classification, not have specific chromosome translocation. GS occurrence in AML: M7 patient was extremely rare. This is the first case report of AML: M7 with GS in the central nervous system.     

Primary cerebellar extramedullary myeloid cell tumor mimicking oligodendroglioma

Extramedullary myeloid cell tumors (EMCTs) are tumors consisting of immature cells of the myeloid series that occur outside the bone marrow. Most of them are associated with acute myelogenous leukemia or other myeloproliferative disorders, and a small number occur as primary lesions, i.e., are not associated with hematological disorders. Occurrence inside the cranium is rare, and there has been only one case of primary EMCT involving the cerebellum reported in the literature. The case we report here is a blastic EMCT occurring in the cerebellum of a 3-year-old boy who had no signs of leukemia or any hematological disorder throughout the entire course. The cerebellar tumor was at first misdiagnosed as an “oligodendroglioma” because of the uniformity and “fried egg” artifact of the tumor cells. The tumor disappeared during chemotherapy consisting of 12 treatments. However, it recurred and metastasized to the cerebrospinal fluid (CSF) shortly after the therapy was completed. A diagnosis of EMCT was suspected because of the presence of immature myeloid cells in the CSF, and was confirmed by anti-myeloperoxidase and anti-lysozyme immunoreactivity of the cerebellar tumor. The patient succumbed 1 year and 3 months after the first presentation of the disease. Received: 6 December 1996 / Revised, accepted: 14 March 1997     

Spinal angiolipoma: case report

Spinal extradural angiolipomas are rare benign tumours, accounting for only 0.14-1.2% of all spinal neoplasms. They are usually localized within the thoracic extradural space of the spinal canal and their common clinical presentation is myelopathy, mainly in the way of a slowly progressive paraparesis and sometimes in an acute form. We report the case of a 85 year old man who complained of bilateral lower limb weakness with sphincter disturbance of acute instauration. The MRI showed a posterior extradural lesion at L1-L2 level that compressed conus medularis and cauda equina. He underwent urgent surgical treatment and we achieved total resection of the lesion via a L1-L2 laminectomy. The pathological examination confirmed the tumour as an angiolipoma.     

Isolated Recurrence of Intracranial Granulocytic Sarcoma Mimicking a Falx Meningioma in Acute Myeloblastic Leukemia

Intracranial granulocytic sarcomas are rare tumors, which are composed of immature granulocytic cells. Although it has been well known that these tumors are associated with acute myeloblastic leukemia (AML), they have been almost always related to bone marrow relapse. However, isolated recurrence of granulocytic sarcoma following complete remission from prior AML is extremely rare, especially in the central nervous system. A 44-year-old male presented with isolated recurrence of granulocytic sarcoma mimicking a falx meningioma two years after complete remission by allogenic peripheral blood stem cell transfusion (PBSCT) in the acute myelomonoblastic leukemia (FAB, M4). Because of depressed mental state and mass effect, total surgical resection was performed. Pathological findings were compatible with the granulocytic sarcoma. There was no evidence of leukemic relapse in the peripheral blood. We suggest that this phenomenon can be explained by the hypothesis that a certain barrier effect such as blood brain barrier might lead to the proliferation of intracranial leukemic cells which metastasized before PBSCT.     

Granulocytic sarcoma of the brain revealed on vascular mode

Granulocytic sarcoma is a rare tumor composed of immature granulocytic cells. Prognosis is poor. The dura and orbits are preferentially involved. An intracranial localization is unusual. We report the case of a 31-year-old man who was admitted with a history of headache and vomiting. The neurological examination revealed hemiplegia. Computed and tomography (CT) and magnetic resonance imaging (MRI) showed a lesion which was interpreted as a meningioma. After surgical resection, pathology examination led to the diagnosis of granulocytic sarcoma. The clinical and radiological features of this case are discussed with reference to other reports in the literature.     

Intracranial myeloid sarcoma presentation in distant acute myeloid leukemia remission

Intracranial myeloid sarcoma (IMS) is a rare central nervous system manifestation of hematopoietic neoplasms of myeloid origin. We report the first case of IMS treatment with an isocitrate dehydrogenase-2 (IDH-2) inhibitor, Enasidenib, following surgical resection, whole-brain radiation, and consolidation Etoposide/Cytarabine therapy. A 42-year-old female was diagnosed with IMS after a 10-year remission of her acute myeloid leukemia (AML). She underwent surgical debulking and had postoperative resolution of her visual symptoms. She received adjuvant radiation and medical management, and continues to show no evidence of recurrence or progression at 17 months postoperatively. This case is notable for an isolated IMS presentation in a patient with a very distant history of AML remission, and without evidence of concurrent bone marrow relapse. The goals of neurosurgical intervention should be symptomatic relief of mass effect and pathological diagnosis, due to the sensitivity of IMS to adjuvant radiation and medical management such as IDH-2 inhibitors.     

Acute leukemia presenting with blasts first found in the cerebrospinal fluid but not in the peripheral blood

Acute leukemia presenting with central nervous system (CNS) signs and symptoms is uncommon and prone to be misdiagnosed. Here, we report nine patients with acute leukemia, including five patients with acute lymphoblastic leukemia (ALL) and four patients with acute myeloid leukemia (AML). These patients presented with symptoms suggestive of involvement of multiple cranial nerves, the spinal cord, and meningeal involvement. Moreover, we found that all these patients unexpectedly showed the presence of blasts in the cerebrospinal fluid (CSF) but not in the peripheral blood despite repeated examinations. Bone marrow examination confirmed the presence of acute leukemia in these patients. Seven patients died within 18 months of diagnosis and two patients developed stable disease. Our findings show a novel presenting feature of acute leukemia and highlight the importance of CSF cytology in the diagnosis of acute leukemia.     

Meningeal involvement in leukemias and malignant lymphomas of adults: Incidence, course of disease, and treatment for prevention

As prognosis has improved over the last several years, an increasing incidence of meningeal involvement has been recognized in adult patients with acute leukemias and malignant lymphomas. In 210 patients evaluated retrospectively, the incidence of meningeal disease was 33% for patients with acute lymphocytic leukemia (ALL), 20% for patients with acute myelogenous leukemia (AML), 22% for patients with non-Hodgkin's lymphomas with an unfavorable histology (NHL), 3% for patients with chronic myelogenous leukemia (CML), and 1% for patients with chronic lymphocytic leukemia (CLL). In most patients, meningeal involvement appeared several months after diagnosis of acute leukemia, often preceding systemic relapse if bone marrow remission had been achieved before. Prophylactic treatment of the CNS was begun in eight patients with ALL or AML after bone marrow remission was achieved. Of these patients, three with ALL and one with AML were free of disease up to 2 years after diagnosis. Methods, benefits, and risks of prophylactic treatment of the CNS for adult patients are discussed in detail.     

脑膜恶性黑色素瘤病三例临床病理及神经影像学特点

目的 探讨脑膜恶性黑色素瘤病的临床、病理及神经影像学特点.方法 对我院收治的经病理确诊的3例脑膜恶性黑色素瘤病患者进行观察,总结其临床、脑脊液细胞学、神经影像学、脑膜组织病理等方面特点.结果 3例患者均以头痛起病,随后出现脑膜刺激征.其中1例皮肤有巨大黑色素痣,1例额部黑痣破溃经久不愈,另一例无皮肤及内脏黑色素瘤,为原发性.3例患者颅脑MRI强化后显示软脑膜及蛛网膜弥漫性较均匀强化并增厚.脑脊液可见大最的异形细胞.脑膜呈黑褐色或深棕色,光镜下肿瘤细胞呈多形性,核大而圆或呈不规则形,胞质丰富,核分裂象可见,胞质内黑色素颗粒聚集,细胞排列紊乱.免疫组织化学分析S-100蛋白、波形蛋白、黑色素瘤抗体HMB-45等胞质反应阳性.结论 脑膜恶性黑色素瘤病临床主要表现为头痛及脑膜刺激征,脑脊液可见大量的异形肿瘤细胞.脑膜病理见肿瘤细胞胞质内黑色素颗粒聚集、瘤细胞排列紊乱.颅脑强化MRI对该病的诊断具有一定价值.     

Leptomeningeal signet-ring cell carcinomatosis presenting with ophthalmoplegia, areflexia and ataxia.

We report a very rare case of occult leptomeningeal carcinomatosis (LC) in whom repeated cytological examination did not show malignant cells in cerebrospinal fluid (CSF) and the primary focus was not discovered by extensive survey. The patient presented with ophthalmoplegia, ataxia and areflexia mimicking Miller Fisher syndrome (MFS) at the initial stage, and later, the clinical profile and laboratory findings including CSF examination simulated tuberculous meningitis. Postmortem autopsy disclosed metastatic signet-ring cell carcinoma infiltrating into cranial nerves and leptomeninges. We would like to emphasize that LC sometimes shows symptoms and signs similar to MFS or tuberculous meningitis.     

Pitfalls in diagnosing limbic encephalitis - a case report

Background – The syndrome of limbic encephalitis (LE) is characterized by subacute onset of temporal lobe epilepsy, loss of short‐term memory, cognitive confusion and psychiatric symptoms. Aim – We report a patient with pharmacoresistant epilepsy who underwent presurgical video‐electroencephalogram (EEG)‐monitoring with normal psychiatric and neuropsychological findings. Methods – Magnetic resonance imaging (MRI) revealed a hyperintense lesion within the right amygdala but no contrast enhancement. Analysis of cerebrospinal fluid (CSF) showed pleocytosis and positive oligoclonal bands, but all tests for neurotropic viruses or borrelia antibodies were negative. Presurgical evaluation identified a right mesiotemporal focus. Results – As a tumour was the most likely differential diagnosis, we performed selective amygdalohippocampectomy of the right hemisphere. Subsequent histopathological examination revealed the surprising diagnosis of LE. As a consequence, tumour screening was initiated and a testicular carcinoma with high anti‐Ma2‐antibody titres was detected. Following surgical and chemotherapeutical treatment, the patient was seizure‐free and Ma2‐antibodies decreased below detection limits. Conclusion – This case report highlights that LE has to be considered even in patients with atypical clinical presentation, i.e. without neuropsychological deficits, if CSF analysis reveals an inflammatory response. When LE is diagnosed, extensive tumour search is mandatory to detect and treat the paraneoplastic origin of LE. Therapeutic strategies of LE include surgical treatment as well as early immunosuppression.     

Cerebrospinal fluid IL-6, TNF-α and MCP-1 in children with acute lymphoblastic leukaemia during chemotherapy

The aim of the study was to investigate the levels of cerebrospinal fluid (CSF) cytokines during chemotherapy of acute lymphoblastic leukaemia (ALL). Examination of 12 ALL child (6 boys and 6 girls) patients evidenced significant increases in interleukin-6 (IL-6) and monocyte chemotactic protein-1 (MCP-1) after induction treatment and significant increases in IL-6, tumour necrosis factor-α (TNF-α) and MCP-1 levels during the consolidation phase, as compared to their values at the time of diagnosis. There were no significant differences in CSF IL-6, TNF-α and MCP-1 concentrations after therapy. Our data suggest that standard ALL treatment may cause a subclinical inflammation and neurotoxicity.     

Platelet functions and clinical effects in acute myelogenous leukemia

Platelets interact with normal peripheral blood cells via adhesion as well as soluble mediators, and platelet released mediators can affect hematopoietic stem and progenitor cells. Interactions may also be involved between platelets and circulating malignant cells, which is suggested by the effects platelets seem to have on metastasis and the various platelet abnormalities observed in various malignant disorders, including acute myelogenous leukemia (AML) and other leukemias. It is only recently that the interactions between platelets and AML cells have been characterized in detail, and studies show that; i) platelets and AML blasts can affect functional characteristic of each other, ii) chemotherapeutic drugs frequently used in AML therapy can alter several platelet functions, iii) the systemic levels of various cytokines are enhanced during AML chemotherapy, including cytokines known to affect both leukemic blasts and platelet activation, and iv) platelet secretion of growth factors are clearly detected in peripheral blood stem cells autografts. In this review we describe platelet interactions with normal leukocytes, normal hematopoietic and leukemic cells and the possible clinical relevance of these interactions in AML.     

Pleomorphic xanthoastrocytoma with intraventricular extension and anaplastic transformation in an adult patient: Case report

Pleomorphic xanthoastrocytoma (PXA) is a rare, low-grade astrocytic tumour that usually occurs in the superficial cerebral hemispheres of children and young adults. Although it has a relatively favourable prognosis, malignant progression of these tumours has been described. Therefore, we present an unusual case of a 54-year-old male with a right, multicystic, parietooccipital tumour extending through the ipsilateral ventricle. After surgical resection, histological examination revealed a lesion with pleomorphic cells, cytoplasmic lipidisation, intensely eosinophilic granular bodies, well-delimitated unique nuclei and focal, positive immunoreactivity for synaptophysin, glial fibrillary acidic protein (GFAP), S-100 protein, vimentin and CD56. Once other tumours, such as giant cell metastatic carcinoma or primary lesion like subependymal giant cell astrocytoma, were ruled out, a final diagnosis of XAP was established. After a follow-up period of 9 months, the patient suffered an extensive and local tumour relapse considered inoperable, with progressive neurological deterioration and radiological findings of malignant progression. The brain biopsy procedure revealed anaplastic changes, including necrosis foci, higher mitotic activity (5×10 high-power fields) and a 10% proliferation index measured by Ki67 labelling. The present case showed intraventricular extension and a more aggressive behaviour, both uncommon in these tumours (similar to anaplastic astrocytoma or glioblastoma multiforme), thus demanding an initial, optimal surgical treatment with close clinical and radiological follow-up, due to the high potential for malignant transformation of XAPs.     

Difficulty in diagnosing chronic meningitis caused by capsule-deficient Cryptococcus neoformans

We report a case of chronic meningitis due to capsule-deficient Cryptococcus neoformans which could not be diagnosed by routine morphological and immunological cerebrospinal fluid (CSF) examination. Repeated CSF examination and culture did not disclose the fungal body, and the cryptococcal antigen tests were always negative. Culture of ventricular fluid showed non-encapsulated cultured cells which were stained positively by indirect immunofluorescence using diluted sera from another patient diagnosed with cryptococcal meningitis. Inoculation of dispersed colonies into mice resulted in encapsulated C neoformans. It is important to suspect capsule-deficient C neoformans when the conventional diagnostic approaches fail to identify the organism or antigens.     

Surgery in the management of primary intracranial germ cell tumors

Surgery plays an important part in the overall management of primary central nervous system (CNS) germ cell tumors. While the general surgical objectives in patients with these neoplasms are similar to those with other types of CNS tumors, to obtain an accurate histopathologic diagnosis and to contribute towards improving patient survival the unique features of germ cell tumors have necessitated novel treatment strategies. Pure germinomas are exquisitely radiosensitive, and prior studies have shown no survival benefit from radical resection of such lesions. However, a significant proportion of CNS GCTs contain admixtures of nongerminomatous GCT (NGGCT) elements and are less responsive to aggressive chemotherapy and irradiation. Biopsy of these malignant GCTs carries the risk of histologic sampling error. Nevertheless, a proportion of NGGCTs produce tumor markers detectable in serum or CSF and may be accurately diagnosed without surgical intervention. Although the role of radical surgical resection has not been definitively demonstrated in the literature, recent data from an international cooperative trial suggest a survival benefit from radical resection of localized NGGCTs. Lastly, increasing experience has supported the role of delayed resective ("second-look") surgery for patients with negative tumor markers but residual radiographic abnormalities after initial chemotherapy. Resection of such lesions has typically yielded necrosis or teratoma, which may be cured by surgical resection, and obviated the need for additional chemotherapy or irradiation.     

A patient with so-called primary reticulum cell sarcoma of the brain with 6.5 years' survival,treated as ‘meningeal leukaemia’

This paper describes the case history of a 43 year-old patient with so-called primary reticulum cell sarcoma of the brain. The CSF contained cells suggestive of leukaemia which, in view of an assumed myelofibrosis, initially seemed also possible to originate from a haematopoietic focus.The patient complained of intermittent violent headaches, which were controlled by spinal taps to release highly cellularised CSF under high pressure and by intrathecal MTX injection, every 4–6 weeks over a period of 4.5 years.In the terminal stage the patient developed paraplegia which, partly on the basis of neuropathological findings, was ascribed to the large accumulated dose of MTX.The tumour proved to be localised in the fornix; the localisation adjacent to the ventricular system made the intermittent cell eruptions in the CSF possible.On the basis of an erroneous diagnostic assumption, a therapy was instituted which resulted in a survival of 6.5 years, which is exceptionally long for a patient with ‘primary reticulum cell sarcoma’ of the brain.     

No chromosomal imbalances detected by comparative genomic hybridisation in a case of fetal immature teratoma

CASE REPORT: We present the first application of comparative genomic hybridisation (CGH) in fetal brain tumours in the case of a fetal immature teratoma. The tumour was discovered in a female fetus at 22 weeks gestation after presentation with significant craniomegaly of such degree that the head size measured by sonography was found to correspond to a gestational age of 37 weeks, and the pregnancy was terminated. RESULTS: Neuropathological examination showed an immature teratoma measuring 1.7 x 1.6 x 0.5 cm and containing tissue from all three germinal layers. CGH was successfully performed and, in contrast to earlier results in both cerebral and extracerebral teratomas also investigated with the same technique, it revealed no chromosomal imbalances. CONCLUSION: This indicates that mono- or trisomies did not have a role in the pathogenesis in this particular case and that a fetal immature teratoma may contain aberrations smaller than the detection threshold of CGH. However, it remains to be seen in larger cohorts whether fetal teratomas follow a different pathogenetic pathway and may be triggered by different molecular events than teratomas occurring in later life.     

Teratoma of the conus medullaris

Intramedullary teratoma is a rare neoplasm. To our knowledge only 10 cases have been reported so far, eight of mature teratoma and two of immature. We present a new case of mature teratoma located in the conus medullaris. After 2 years increasing complaints and worsening neurologic deficits, the neoplasm was discovered by magnetic resonance imaging and was completely resected surgically. Similar cases are reviewed and the clinical, radiological, surgical and morphological aspects of this lesion will be discussed. In the majority of cases, the tumour has an indolent growth rate and recovery after resection is good.