Oligodendroglioma metastasizing to cervical lymph node: Rare entity diagnosed on fine-needle aspiration cytology.

Extra neural metastasis of central nervous system oligodendroglioma is very rare. Oligodendroglioma is the seventh most frequently occurring neoplasm of central nervous system (CNS) with metastasis outside the CNS. According to literature, presence of metastasis in CNS was most frequently detected in patients of glioblastoma (41.4%), medulloblastoma (26.7%), ependymomas (16.4%), astrocytoma (10.3%) and oligodendroglioma (5.27%). A 38-year-old male patient presented with loss of vision and swelling on left side of neck since last 1 week measuring 3 x 2 cm. He was operated for brain tumor 7 years back, which was diagnosed as oligodendroglioma. Ultrasound sonography revealed multiple hypoechoic lymph nodes in bilateral cervical region largest measuring 4.5 x 1.9 cm in left submandibular region. FNA of left submandibular lymph node was done, which revealed deposits of poorly differentiated malignancy. Cell block was prepared for carrying out ancillary studies which showed positivity for glial fibrillary acidic protein (GFAP), S-100 and negativity for cytokeratin (CK), epithelial membrane antigen (EMA), LCA and progesterone receptor (PR). Based on previous history of oligodendroglioma, cytological and immunohistochemistry (IHC) findings a diagnosis of metastatic oligodendroglioma was made. Metastasis of oligodendroglioma to cervical lymph node should also be considered as one of the differential diagnoses. Diagnosing metastatic CNS tumor is extremely challenging for pathologists. It is essential to have the clinical information of a previous CNS tumor, including the histologic type and immunophenotype. Besides common malignancies of cervical lymph node, we should also think of CNS metastasis so that patient management will be early and proper.

Symptomatic Spinal Seeding Metastasis of a Low-grade Oligodendroglioma.

Extracranial metastases from primary brain tumours are mostly caused by high-grade tumours. Metastases from low-grade intracranial tumours are much rare and usually asymptomatic. We present a case of a symptomatic spinal cord compression with intradural extramedullary and diffuse leptomeningeal infiltration observed approximately 51 months after the first diagnosis of a 52-year male patient with WHO Grade 2 oligodendroglioma with temporoparietal localisation. This patient, who had the complaint of weakness in the lower extremity, was operated on due to a thoracic intradural extramedullary mass. The result of the pathological examination came out as WHO Grade 2 oligodendroglioma, and radiotherapy was planned for this seeding metastasis. The patient who experienced refractory seizures died before his radiotherapy treatment was completed. It should be kept in mind that spinal metastases may also be seen in low-grade intracranial tumours without malignant transformation as in the present case. Key Words: Spinal seeding, Spinal metastases, Low-grade oligodendroglioma.

Bone metastases from a 1p/19q codeleted and IDH1-mutant anaplastic oligodendroglioma: a case report.

BACKGROUND: Oligodendroglioma is a rare type of primary brain tumor which, like other malignant gliomas, metastasizes very rarely even when in high-grade form. CASE REPORT: A 36-year-old white man diagnosed 29 months previously as having 1p/19q codeleted anaplastic oligodendroglioma presented bilateral cruralgia and lower limb motor deficits. A computed tomography scan showed multiple osteoblastic bone lesions. The presence of oligodendroglial cells was revealed by bone marrow biopsy and confirmed by immunohistochemical analyses. A positon emission tomography-computed tomography scan confirmed the exclusive involvement of bones. CONCLUSION: This case joins less than 20 other reported cases of oligodendroglioma bone marrow metastasis, and is one of only a handful of cases of diffuse bone metastases beyond the axial skeleton. To the best of our knowledge, the early relapse of 1p/19q codeleted anaplastic oligodendroglioma with this distribution of metastases has never been described in the literature.

Scalp Metastasis of Anaplastic Oligodendroglioma.

BACKGROUND: Scalp metastases from anaplastic oligodendroglioma (AO) are extremely rare and mostly involve intracranial recurrence or widely metastatic disease. Here we describe an exceptional case of histopathologically proven scalp metastasis of AO 6 years after surgical resection and postoperative adjuvant radiation. CASE DESCRIPTION: A 42-year-old woman presented with several months of progressive headache and dizziness. Preoperative magnetic resonance imaging (MRI) showed an irregular enhancing lesion in the left frontal lobe extending to the ependymal surface. Left frontal craniotomy was performed through a coronal approach, and gross total resection was achieved. Pathologic examination confirmed a World Health Organization grade III AO. The patient subsequently received 60 Gy of external beam radiotherapy in 30 fractions over 6 weeks. During 8 years of follow-up, the patient remained symptom free, and no evidence of intracranial recurrence was found. However, 6 years after intracranial tumor resection, the patient noticed a subcutaneous mass in her right frontal scalp, which was the site contralateral to her craniotomy. MRI revealed a homogeneously marked enhancing nodular lesion in the subcutaneous tissue of the right frontal scalp without intracranial recurrence. Gross total resection was performed, and the pathologic findings, which identified the mass as an AO, were consistent with those of the primary left frontal tumor. CONCLUSIONS: This study presents a rare case of long-term AO scalp metastasis without intracranial recurrence. Intraoperative seeding and longer survival for oligodendroglial tumors may cause this rare entity. Optimal surgical strategies and standard operative procedures can promote the prevention of iatrogenic seeding.

Leptomeningeal metastases in glioma: The Memorial Sloan Kettering Cancer Center experience.

OBJECTIVE: To perform a retrospective analysis examining the incidence and prognosis of glioma patients with leptomeningeal disease (LMD) at Memorial Sloan Kettering Cancer Center over a 15-year period and correlate these findings with clinicopathologic characteristics. METHODS: We conducted a retrospective review of glioma patients with LMD at Memorial Sloan Kettering Cancer Center diagnosed from 2001 to 2016. Patients were identified through a keyword search of their electronic medical record and by ICD-9 codes. RESULTS: One hundred three patients were identified with disseminated LMD and 85 patients with subependymal spread of disease, 4.7% of all patients with glioma. These cohorts were analyzed separately for time to development of disseminated LMD/subependymal LMD, median overall survival, and survival from LMD diagnosis. Patients were pooled for subsequent analyses (n = 188) because of comparable clinical behavior. LMD was present at glioma diagnosis in 10% of patients. In the remaining 90% of patients diagnosed at recurrence, time to LMD diagnosis, survival after LMD diagnosis, and overall survival varied by original histology. Patients with oligodendroglioma had a median survival of 10.8 (range 1.8-67.7) months, astrocytoma 6.5 (0.1-28.5) months, and glioblastoma 3.8 (0.1-32.6) months after LMD diagnosis. In addition, we found that treatment of LMD was associated with superior performance status and increased survival. CONCLUSION: Patients with LMD diagnosed at relapse may not have decreased overall survival as compared to historical controls with parenchymal relapse and may benefit from treatment.

Successful treatment of a BRAF V600E-mutant extracranial metastatic anaplastic oligoastrocytoma with vemurafenib and everolimus.

BACKGROUND: Extracranial metastasis is a rare phenomenon of anaplastic oligoastrocytoma. When patients progress after comprehensive treatment, there is often no effective treatment. Rapid development of gene detection technology makes precision treatment of glioma possible. PATIENT AND METHODS: A 22-year-old girl was firstly diagnosed with anaplastic oligoastrocytoma WHO grade III-IV in 2014, and progressed rapidly after chemoradiotherapy in multiple extraneural lesions in 2016. She was expected to have a short life and Next-Generation Sequencing (NGS) was applied. RESULTS: Mutation of BRAF (V600E) was reported by 1st NGS and oral vemurafenib stabilized her disease for 6 months. PIK3CA was reported by 2nd NGS after her progression of vemurafenib. The oral administration of everolimus together with vemurafenib stabilized her disease for another 6 months. However, the patient died due to the rapid progression of the disease on 24 February 2018. CONCLUSION: We successfully treated a BRAF V600E-mutated anaplastic oligoastrocytoma with multiple extraneural metastases with vemurafenib and everolimus. For late-staged patients who have no clear and effective treatment plan, NGS may serve as an effective option.

[A Case of Bone Marrow Metastasis of Oligodendroglioma with IDH Mutation and 1p/19q Codeletion].

A 71-year-old woman was transferred to our hospital, complaining of a seizure for the first time. A tumor was detected in the right frontal lobe, and a craniotomy was performed with a partial tumor resection. The pathological diagnosis was oligodendroglioma with IDH mutation and 1p/19q codeletion, and irradiation therapy was performed. Six months later, the patient's lactate dehydrogenase(LDH)level elevated remarkably, and the fluoro-deoxyglucose-positron emission tomography/computed tomography showed abnormal uptake in multiple bone marrow locations. Bone marrow aspiration was performed, and the pathological diagnosis was oligodendroglioma metastasis. The patient was given two cycles of chemotherapy with temozolomide(TMZ), and her LDH level reduced to normal. After a few months, the LDH level elevated again, so we gave her two more cycles of TMZ;however, her LDH level did not change. Thereafter, the patient was hospitalized because of paraplegia, which started a few days prior, and right lower jaw swelling. Her CT and magnetic resonance imaging showed metastasis to the thoracic vertebrae and right mandibular bone. Irradiation therapy was performed to these locations, and the patient was given chemotherapy using nimustine(ACNU), procarbazine, and vincristine(PAV). Her LDH levels reduced temporarily, but elevated again. The patient deteriorated slowly and died 20 months after she presented with a seizure. Oligodendroglioma with extracranial metastasis is extremely rare, and this case report is the 68th report. The chemotherapy approach with TMZ or PAV/PCV may be effective against oligodendroglioma metastasis to the bone marrow.

Bone Marrow Metastases from a 1p/19q Co-deleted Oligodendroglioma - A Case Report.

BACKGROUND: Metastasis of oligodendroglioma outside of the central nervous system (CNS) is a very rare event. CASE: We describe in detail the clinical story of a 50-year-old woman diagnosed with profound pancytopenia and signs of extramedullary hematopoiesis caused by diffuse bone marrow replacement by a metastatic oligodendroglioma. RESULTS: Upon development of pancytopenia, a diagnostic bone marrow examination revealed the presence of metastatic oligodendroglial cells. No others sites of malignant dissemination were found outside the CNS. Despite best supportive care, the patient died three weeks after diagnosis of myelophthisis. CONCLUSION: Extracranial metastases from CNS oligodendroglioma are a very rare event but can be the cause of bone marrow failure.

Brain gliomas presenting with symptoms of spinal cord metastasis.

Three patients with brain gliomas (aged 41, 37, and 43 years) presented spinal cord symptoms as first neurological presentation (two cases) or at anaplastic progression (one case). Histologically, two cases were anaplastic (WHO III) astrocytomas and one anaplastic (WHO III) oligodendroglioma. The spinal surgery consisted of partial tumor resection in two cases with localized spinal cord metastasis, and tumor biopsy in another with diffuse spreading to the conus and cauda. Spinal irradiation was performed in one case. The time interval between the spinal surgery and the appearance of brain symptoms was very short (1 month or less). Two patients underwent brain surgery (tumor resection in one and stereotactic biopsy in another). The survival time was very short (2 and 3 months) in the two patients with anaplastic astrocytoma, whereas the patient with anaplastic oligodendroglioma survived 1 year after the spinal surgery. Brain gliomas may exceptionally present with symptoms of a spinal cord metastasis. The magnetic resonance imaging finding of a spinal cord enhancing lesion, particularly if associated with root enhancement, should suggest the presence of a brain glioma. In cases with a localized spinal lesion, an early spinal surgery is advised for both diagnosis and decompression of the nervous structures. However, the clinical outcome is poor and the survival time is short.

Secondary oligodendroglioma after postoperative irradiation for medulloblastoma: a case report and review of the literature.

Medulloblastoma, a malignant, invasive embryonal tumor of the cerebellum, occurs most often in children. It has high metastatic potential and is usually treated by aggressive multimodal therapy, including surgery, chemotherapy and craniospinal irradiation. Multiple secondary tumors have been reported following craniospinal irradiation. It is rare with the occurrence of oligodendroglioma after irradiation. In this report, we described a patient with secondary oligodendroglioma after postoperative craniospinal irradiation for medulloblastoma.

Occipital anaplastic oligodendroglioma with multiple organ metastases after a short clinical course: a case report and literature review.

BACKGROUND: It is generally believed that malignant gliomas never metastasize outside the central nervous system (CNS). However, the notion that oligodendrogliomas (OGDs) cells cannot spread outside CNS is being challenged. METHODS: We described in detail the clinical story of one patient with anaplastic OGD, which metastasized to lymph nodes, bone marrowand bones Genetic analyses included detection of 1p and 19q chromosomal arms, methylation status of MGMT promoter, and PTEN exon mutations. A search of worldwide literature was conducted for reports of metastatic OGDs using NCBI-PubMed, with the keywords "extracranial", "extraneural", "oligodendroglioma", "oligodendrogliomas", "metastatic", "metastasis", and "metastases", in different combinations. RESULTS: An open biopsy of the infiltrated bones in our patient revealed that malignant cells had replaced the patient's marrow. Moreover, the diagnosis of multiple-organ metastases of anaplastic OGD was confirmed based on immunohistochemical staining. Genetic analyses showed that the tumors originated from previously resected brain lesions. None of the lesions had 1p and 19q deletions, but hypermethylation of MGMT promoter, and the G → A transversion at codon 234 of PTEN exon 2 were detected. Literatures review yielded 60 reports of metastatic OGDs from 1951 to the present, which with our patient makes 61 cases. Concerning these 61 patients, there were 110 infiltrated sites correlated closely with primary OGDs. The most frequent metastatic sites were bone and bone marrow (n = 47; 42.7%), lymph nodes (n = 22; 20.0%), liver (n = 7; 6.4%), scalp (n = 6; 5.5%), lung (n = 6; 5.5%), pleura (n = 4; 3.6%), chest wall (n = 3; 2.7%), iliopsoas muscle (n = 2; 1.8%), soft tissue (n = 2; 1.8%), and parotid gland (n = 2; 1.8%). CONCLUSIONS: Extracranial metastases in anaplastic OGD are very rare but they do occur; bone and bone marrow may be the most common sites. Detection of certain molecular markers such as deletion of 1p and 19q chromosomal arms, hypermethylation of MGMT promoter, and characteristic PTEN exon mutations may help differentiate subtypes which are more prone to extracranial metastases. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8749838611478560.

Leukemia-like onset of bone marrow metastasis from anaplastic oligodendroglioma after 17 years of dormancy: an autopsy case report.

Extraneural metastases from primary brain tumors are extremely rare. We present an autopsy case that displayed a very late and unique pattern of metastasis from an anaplastic oligodendroglioma. The patient was a 74-year-old woman who was disease free for 17 years after resection of the primary oligodendroglioma. She was subsequently admitted to a hospital for heart failure where her bone marrow was found to be completely infiltrated with tumor cells, eventually resulting in disseminated intravascular coagulation. The onset was like leukemia, but the "blast-like" cells were different from leukemic cells, and the diagnosis was difficult until autopsy. After her death, a review of her past medical history and comprehensive analysis of her primary brain tumor and aspiration biopsy/autopsy bone marrow samples with glial immunohistochemical markers, fluorescence in situ hybridization examination, and immunohistochemical/sequencing analyses of mutant IDH1 revealed the accurate diagnosis. The metastatic tumor in her bone marrow was finally diagnosed as bone metastasis from the primary anaplastic oligodendroglioma. Although metastatic oligodendroglioma is very rare, it should be noted that this condition displays a propensity for bone and bone marrow and can present with features similar to those of leukemia after a long latency period.

Fine-needle aspiration cytology of metastatic oligodendroglioma: case report and literature review.

BACKGROUND: Systemic metastasis of a glial tumor is a rare event. However, metastatic cases are anticipated to increase due to prolongation of survival as a result of the development of new treatment modalities. The possibility of metastasis should be considered in patients with a history of a glial tumor rather than a second primary tumor. Fine-needle aspiration cytology is one of the diagnostic procedures primarily applied for confirmation of metastasis in cases with a known primary focus. Therefore, comprehensive knowledge of diagnostic cytomorphologic findings is required in these cases. CASE REPORT: We report a young woman with oligodendroglioma metastasizing to the cervical lymphatic chain 5 years after initial diagnosis. Fine-needle aspiration cytology revealed a highly cellular smear with dispersed single cells and loosely cohesive cell clusters showing rosette-like features on a clean background. The relatively monotonous tumor cells were small sized and had round nuclei with moderate anisonucleosis and scant cytoplasm without extensions. Diagnostic confirmation was made by excisional biopsy and demonstration of 1p19q codeletion on tissue section by fluorescence in situ hybridization. CONCLUSION: A brief review of the literature with an emphasis on the cytologic features of metastatic oligodendroglioma and differential diagnosis with respect to other metastatic small round cell tumors is provided.

Paraplegia due to drop metastases from anaplastic oligodendroglioma.

In this article, we report on a rare case of spinal seeding from a cerebral anaplastic oligodendroglioma presenting with signs of medullar compression. We discuss the prevalence, mechanisms and imaging findings of spinal seeding in various gliomas. A suitable clinical treatment and follow up for these patients is suggested.

Breast metastasis of anaplastic oligodendroglioma: a case report.

Extracranial metastasis of primary brain tumors is rarely observed. Of all brain malignancies, glioblastomas, medulloblastomas and astrocytomas metastasize most frequently. Metastasis of oligondendroglioma is rare. We present a case of breast metastasis in a 58-year-old man with an anaplastic oligodendroglioma.

Extracranial metastases of anaplastic oligodendroglioma.

Extracranial metastasis of a malignant glioma is rare, possibly due to the lack of lymphatic drainage in the brain and because these tumors are unable to penetrate blood vessels. Extracranial metastasis of an anaplastic oligodendroglioma (ODG) is exceptionally rare. We present a 55-year-old male patient with diffuse extracranial metastases from a temporal anaplastic ODG, 11 months after cranial surgery. Anaplastic ODG, may spread to the other parts of the body. If patients with these tumors have neck or back pain, spinal metastasis should be included in the differential diagnosis and further investigated.

Bone marrow metastases from anaplastic oligodendroglioma presenting with pancytopenia and hypogammaglobulinemia: a case report.

We report the case of a 40-year-old man whose bone marrow metastases occurred 57 months after the initial diagnosis and 9 months after completing radiotherapy for an anaplastic oligodendroglioma. Four months before the demonstration of visceral metastases was obtained by bone marrow biopsy, the patient developed diffuse bone pain, pancytopenia, hypercalcemia, and panhypogammaglobulinemia. These abnormalities and other clinical signs of extracranial dissemination of the primary brain tumor were initially unrecognized until the patient was admitted with the suspicion of a nonsecretory multiple myeloma. We also briefly review the factors predisposing these tumors to spread outside the CNS, albeit rarely, and discuss the clinical implications of a misdiagnosis of extracranial invasion by anaplastic oligodendroglioma, whose chemosensitivity has been definitively demonstrated.

Detection of IDH1 mutations in gliomatosis cerebri, but only in tumors with additional solid component: evidence for molecular subtypes.

The current WHO classification of brain tumors defines gliomatosis cerebri (GC) as an extensively infiltrating astrocytic glioma involving at least three cerebral lobes. The relation of GC to diffuse astrocytomas and glioblastoma is uncertain. Due to malignant biological behavior, GC is allotted to WHO grade III. Recent reports showed IDH1 mutations in astrocytic and oligodendroglial tumors WHO grades II and III and in secondary glioblastomas with a frequency of up to 90%, whereas IDH1 mutations occurred in only 5% of primary glioblastomas. Here, we examined the frequency of IDH1 mutations in 35 GC samples by direct sequencing, derived cleaved amplified polymorphic sequence analysis and immunohistochemistry. We identified IDH1 mutations in 10/24 (42%) cases, which also included a solid tumor portion (type 2 GC), but not in 11 "classical" cases without solid tumor mass (type 1 GC). TP53 mutations were revealed in two type 2 GC, but not in any type 1 GC, while combined chromosomal losses of 1p and 19q were not found at all. Our data suggest that GC consists of two histological/molecular subtypes, type 1 being clearly distinct from diffuse astrocytoma, and type 2 sharing features with diffuse astrocytoma.