Extramedullary Mass Research Articles

Mother and daughter with a SMARCE1 mutation resulting in a cervical clear cell meningioma at an identical location: illustrative cases.

A rare meningioma subtype is a clear cell (CC) meningioma, which can be associated with a SMARCE1 gene mutation. Manifestation of a CC meningioma in the cervical spine is unusual. In the current case, both mother and daughter present with a CC meningioma at an identical cervical location. A 67-year-old patient with an intradural extramedullary mass at the level of C5 presented with progressive myelopathy. The mass was resected through a ventral approach by a two-level corpectomy with an expandable cage and instrumentation. The daughter of this patient appeared to have had an intradural extramedullary mass at C5 at the age of 20, which was resected through a posterior approach. Pathological investigation of both tumors revealed CC meningioma. Genetic testing of the daughter revealed a SMARCE1 mutation. It is of major importance to consider a SMARCE1 mutation in elderly presenting with a CC meningioma, which is still uncommon in current practice. This could lead to timely diagnostics in the succeeding generation. Complete resection of a CC meningioma is important because of the high recurrence rate. Routine follow-up should therefore be performed in the postoperative period. An anterior approach should be considered for a ventral cervical CC meningioma.

Clinical characteristics, pathology features and outcomes of pediatric myeloid sarcoma: A retrospective case series.

Myeloid sarcoma (MS) is a rare extramedullary mass with myeloid expression, which is easy to be missed and misdiagnosed, especially in the pediatric population. We analyze the clinicopathological characteristics, immunophenotypic, cytogenetic, and molecular studies, therapeutic approaches, and outcomes, to optimize the management of such patients. A retrospective, single-center, case series study of eleven children diagnosed with MS by pathology was performed. The male-to-female ratio was 8:3, and the median age at diagnosis was 7 years. The most commonly involved sites were the skin and orbital region, followed by lymph nodes, central nervous system, and testis. Seven cases (64%) with Class I-MS and four cases (36%) presented as Class II-MS. Immunohistochemically, MPO and CD117 were the most commonly expressed markers, followed by CD33, CD43, CD34, CD68, and lysozyme. Chromosomal abnormalities were detected in 4 patients. Two patients had the presence of deleterious mutations (FLT3, ASXL, KIT, and DHX15) on molecular detection. Ten patients were treated with chemotherapy based on AML regimens. The median follow-up time was 33.5 months in eleven patients. Two patients relapsed, one died, and one lost to follow-up. The 2-year overall survival (OS) rate estimated by Kaplan-Meier curves was 90.9% ± 8.7%, and the event-free survival (EFS) rate was 64.9% ± 16.7%. MS diagnosis is usually challenging. Adequate tumor biopsy and expanded immunohistochemistry are necessary for the correct diagnosis of MS. Early and regular systemic chemotherapy promises long-term survival.

Drop foot and adult onset tethered cord syndrome caused by filum terminal ependymoma: A case report

Introduction: Filum terminal is a fibrous tissue to stabilize the position of the spinal cord, located in both intradural and extradural sections anchored caudally to the coccyx. Tumors located at the filum terminal are a rare case. Filum terminal does not have any spinal cord component, but tumours in that area can directly cause a significant neurological disorder. Case Presentation: Presenting 43 years old man with chief complaints of drop left foot and tethered cord syndromes. MR Image revealed 9,4x10mm intradural extramedullary mass at L1 with L1-L5 disc desiccation. The patient was untethered by laminectomy and underwent total mass excision. The sample was analyzed pathologically, and the results matched myxopapillary ependymoma. Pain sensation and sensory disturbance immediately improved after surgery. The urinary disorder also gradually improved. The patient was discharged without any urinary problems. After six months of follow-up, painless, mild left lower drop foot and mild gait disturbance was found without residual urinary and other neurological disorder. Conclusion: Filum terminal ependymomas need to be considered for differential diagnoses in patients with drop foot and adult-onset tethered cord syndromes.

AML-034 Granulocytic Sarcoma Disguised as Lymphoblastic Lymphoma

Acute myeloid leukemia (AML) is a malignant clonal disease of hematopoietic stem cells affecting the blood and bone marrow. Granulocytic sarcoma (chloroma) is a rare disease characterized by an extramedullary myeloid cell mass with or without bone marrow infiltration. We report a case of AML presented with granulocytic sarcoma causing cord compression. An 18-year-old male presented in January 2022 with acute onset and progressive low backache and lower limb weakness with no history of associated trauma. The condition was followed one week later by urine and stool incontinence. Clinical examination revealed motor and sensory impairment with bladder and bowel dysfunction. Pan CT scan showed an anterior mediastinal amalgamated lymph node with central necrosis and heterogeneous enhancement measuring 3.5×6 cm. MRI study detected a dorso-lumbar intradural extramedullary lesion 5×8×6 cm and a heterogeneously enhancing pre-sacral soft tissue mass measuring 3.2×7×6 cm. Biopsy and pathological examination of the dorsal mass showed infiltration by sheets and aggregates of malignant round cell tumor with diffuse, positive reaction of the tumor cells by LCA, CD99, PAX5, and BCL2 and 80%-90% of the cells positively stained by Ki-67, a picture suggestive of B-cell lymphoblastic lymphoma. A complete blood count revealed the following: white blood cell count 6.5×109/l, absolute neutrophil count 2.4×109/l, hemoglobin 11.9 g/dl, mean corpuscular volume 88.6 fl, platelets 217×109/l. Along with further immunohistochemical assessment of the dorsal lumbar mass, bone marrow examination showed 70% of blast cells positively stained for MPO, CD117, CD33, CD34, HLADR, and CD58 and negatively stained for TdT, CD79, cCD3, CD22, CD19, CD10, and CD20 with further immune staining of the spinal mass. The picture was consistent with AML. Treatment commenced, and the patient achieved complete remission following 2 induction therapies with a "7+3" regimen. On the clinical level, the patient showed noticeable improvement in sensation and motor power and was under high-dose cytarabine while the article was written. Myeloid sarcoma is a rare tumor consisting of immature granulocytic cells and is frequently misdiagnosed particularly in the absence of clinical suspicion of myeloid malignancies. Competent diagnostic clinical, laboratory, and radiological imaging of granulocytic sarcoma are crucial for proper management.

AML-072 Myeloid Sarcoma Experience from Tertiary Care Centre of Western India

In rare cases, hematological malignancies can manifest as an extramedullary malignant myeloid precursor cell mass. Myeloid sarcoma (MS) is a type of leukemia that can be acute or chronic. At our institute, we will discuss the clinicopathological features and treatment responses of patients with MS. From January 2010 to December 2015, we described the clinicopathological features and treatment responses of 31 MS patients at the Medical Oncology Department of Gujarat Cancer Research Institute in Ahmedabad, Gujarat, India, as well as the relevant literature. MS patients were given systemic chemotherapy using acute myeloid leukemia (AML)-like regimens only or local treatment (radiation, surgery) with or without systemic chemotherapy using AML-like regimens. This study included 31 patients ranging in age from 6 to 68 years (median: 32 years; mean: 35.8 years). There were 15 male and 16 female patients with a ratio of 0.9:1. MS occurrence was most common at the lymph nodes (N=7, 22.6%), followed by the bones (N=5, 16.13%), orbit (N=5, 16.13%), and reproductive organs (N=3, 9.70%). MS was associated with AML in 5 patients (16.13%) and chronic myeloid leukemia in 5 patients (16.13%), and 21 patients (67.74%) had de novo isolated MS. Twelve patients (38.71%) were treated with surgery and/or radiotherapy or chemotherapy (SRC) and 19 (61.29%) with chemotherapy (C). Sixteen patients (51.61%) achieved complete remission (CR), 10 (32.26%) achieved partial remission (PR), and 5 (16.13%) had progression. After treatment, the number of patients who achieved CR was lower in the C group (N=8, 42.11%) than in the SRC group (N=8, 66.67%) (P=0.035). Two patients in the SRC group and 4 in the C group died during the follow-up period (P=0.72). Survival rates for the SRC and C treatment groups were 83.3% and 78.9%, respectively (P=0.0328), with both groups lasting 36 months. To diagnose MS, histopathology, immunohistochemistry, and imaging must be used in tandem. As soon as possible, induction chemotherapy or a tyrosine kinase inhibitor (imatinib) should be administered. Symptomatic lesions or tumors causing organ obstruction are treated with surgery and/or radiotherapy. To describe the characteristics of MS and the role of targeted treatment, prospective controlled trials are required.

Meningioma – A spectrum of varied case presentations: A case series

Meningiomas are most common primary intracranial neoplasm. They arise from the arachnoid cap cells. They were previously divided into WHO grade I, grade II and grade III depending on their histological features and clinical features. However, in the new WHO 2021 updates it has been assigned a single group. Meningiomas may arise in the brain and spinal cord. An integrated approach based on clinical examination, history, radiology and histopathology aids in diagnosis. Herein we present a case series of a spectrum of clinico-pathological presentations of meningiomas. A 35-year-old female presented with headache. After clinical examination and on CT scan an intracranial lesion was noted. Craniotomy was performed and tissues examination was consistent with meningothelial meningioma. A 55-year-old female presented with pain in the limbs associated with weakness to the orthopaedics clinic. On neurological examination there was loss of power in the limbs. MRI spine revealed a round extramedullary intradural mass at T7 level. Laminectomy was done and histopathological examination showed features of Psammomatous meningioma, based on histomorphology and immunohistochemistry.A 32-year-old female presented to the Surgery clinics with dizziness and headache which was progressive and not responding to medications. MRI revealed an ovoid enhancing mass involving left parieto-occipital region, with a provisional diagnosis of meningioma. A report of Transitional meningioma was given based on histomorphology and immunohistochemistry. A 40-year-old female presented to the ophthalmic clinic with complaints of diplopia and disturbances in gait. Fundus examination revealed disc oedema and retinal vessel narrowing. MRI revealed large ovoid extra axial mass, with a provisional diagnosis of meningioma. A report of Secretory meningioma was signed out on histomorphology and immunohistochemistry.

Luspatercept, a two-edged sword in beta-thalassemia-associated paravertebral extramedullary hematopoietic masses (EHMs).

Paravertebral extramedullary hematopoietic masses (EHMs) account for up to 15% of extramedullary pseudotumors in beta-thalassemia (BT) and are most likely related to compensatory hematopoiesis. In most cases, pseudotumors are incidentally detected, as the majority of patients are asymptomatic. Since June 2020, luspatercept is approved for the treatment of patients with BT who require regular red blood cell transfusions. Data addressing the safety and efficacy of luspatercept in patients with BT-associated EHMs are pending. To date (May 2022), paravertebral EHMs were observed in two asymptomatic patients out of currently 43 adult patients with BT registered at the Adult Hemoglobinopathy Outpatient Unit of the University Hospital Essen, Germany. In one of them, a paravertebral EHM was diagnosed more than 10 years prior to referral. Throughout observation time, treatment with luspatercept was associated with a clinically significant reduction in transfusion burden while allowing to maintain a baseline hemoglobin concentration of ≥10 g/dL aiming to suppress endogenous (ineffective) erythropoiesis associated with BT. Considering the rarity of paravertebral EHMs in BT, luspatercept might potentially represent a novel therapeutic option for these often-serious disease-associated complications. However, appropriate follow-up investigations are recommended to detect (early) treatment failures secondary to an undesired luspatercept-associated erythroid expansion.

Incidence, clinical characteristics, and prognostic nomograms for patients with myeloid sarcoma: A SEER-based study.

BackgroundMyeloid sarcoma (MS) is a rare hematological tumor that presents with extramedullary tumor masses comprising myeloid blasts. A controversial issue is whether MS involving normal hematopoietic sites (liver, spleen, and lymph nodes) should be excluded in future studies. We aimed to compare MS characteristics and outcomes involving hematopoietic and non-hematopoietic sites and construct a prognostic nomogram exclusively for the latter.MethodsData from patients diagnosed with MS between 2000 and 2018 were collected from the Surveillance, Epidemiology, and End Results (SEER) database. According to the primary site, patients were classified as having MS involving hematopoietic sites (hMS) or non-hematopoietic sites (eMS). Clinical characteristics and survival outcomes were compared between the two groups using Wilcoxon, chi-square, and log-rank tests. Cox regression analysis was used to identify eMS prognostic factors to establish prognostic nomograms. The models’ efficiency and value were assessed using receiver operating characteristic (ROC) curves, calibration curves, and decision curve analysis (DCA).ResultsIn total, 694 patients were enrolled, including 86 with hMS and 608 with eMS. There were no sex, race or marital status distribution differences between the two groups. Patients with eMS had better overall and cancer-specific survival rates than those with hMS. Additionally, prognostic factor effects differed between the two groups. Patients with eMS were randomly divided into the training (number of patiens, n=425) and validation cohorts (n=183). Age, first primary tumor, primary site, and chemotherapy were used to establish nomograms. The C-index values of overall survival (OS) and cancer-specific survival (CSS) nomograms were 0.733 (validation: 0.728) and 0.722 (validation: 0.717), respectively. Moreover, ROC, calibration curves, and DCA confirmed our models’ good discrimination and calibration ability and potential clinical utility value.ConclusionOur study described the differences between patients with eMS and those with hMS. Moreover, we developed novel nomograms based on clinical and therapeutic factors to predict patients with eMS’ 1-, 3- and 5-year survival rates.

Plasmablastic myeloma in Taiwan frequently presents with extramedullary and extranodal mass mimicking plasmablastic lymphoma.

Plasmablastic myeloma (PBM) is a blastic morphologic variant of plasma cell myeloma with less favorable prognosis than those with non-blastic morphology. PBM is rare, without clear-cut definition and detailed clinicopathologic features in the literature. PBM may mimic plasmablastic lymphoma (PBL) as they share nearly identical morphology and immunophenotype. Using the criteria of ≥ 30% plasmablasts in tissue sections, we retrospectively recruited PBM cases and analyzed their clinical, imaging, and pathologic findings, with emphasis on extramedullary involvement. We performed immunohistochemistry, in situ hybridization for Epstein-Barr virus (EBER), and fluorescence in situ hybridization (FISH) for lymphoma- and myeloma-associated genetic alterations. Of the 25 recruited cases, 15 (60%) had extramedullary involvement, which occurred as initial presentation in nine cases. The most common extramedullary sites were soft tissue and/or skin (10/15, 67%), followed by pleural effusion, the lungs, and lymph nodes. Immunohistochemically, tumor cells expressed MYC (74%; 17/23), CD56 (56%; 14/25), and cyclin D1 (16%; 4/25), while CD117 was all negative (n = 25). Of the 20 cases stained with p53, four (20%) cases were diffusely positive, and the remaining 16 cases showed a heterogeneous pattern. EBER was negative in all 24 cases examined. Of the 13 cases examined with FISH, the genetic aberrations identified included del(13q14)(92%; 12/13), gain of chromosome 1q (90%; 9/10), loss of chromosome 1p (60%; 6/10), IGH-FGFR3 reciprocal translocation (23%; 3/13), rearranged MYC (15%; 2/13), and rearranged CCND1 (8%; 1/13), while there were no cases with TP53 deletion (n = 10) or rearrangement of BCL2 (n = 13) or BCL6 (n = 13). The prognosis was dismal regardless of the presence or absence of extramedullary involvement. In conclusion, PBM in Taiwan frequently presented as extramedullary and extranodal lesions, particularly in soft tissue and/or skin, mimicking PBL. FISH for targeted genetic alterations such as del(13q14), gain of chromosome 1q, loss of chromosome 1p, and IGH-FGFR3 might be helpful for the differential diagnoses. Larger studies are warranted to investigate the genetic alterations between PBM and PBL.

Castleman Disease masquerading as the lumbosacral neurofibroma with excellent response to rituximab

Castleman's disease is an uncommon, lymphoproliferative disease. Its etiology is unknown. Spine involvement is very scant in Castleman's disease. The authors report on the case of a 50 year-old man who referred with saddle anesthesia and low back pain from 2.5 years ago. A CT scan showed an extra medullary mass at S1-S2 level of the spinal canal without bone involvement. The signal specs on MRI were resembling to signal of neurofibroma .The patient underwent needle biopsy. Histological examination confirmed the diagnosis of Castleman disease, hyaline-vascular type. He received rituximab and pain was dramatically relieved. Although rare, Castleman disease should be considered in the differential diagnosis of an extramedullary mass.

Bilateral Myeloid Sarcoma of the Breast: A Case Report With Radiological and Pathological Correlation

Myeloid sarcoma is a solid extramedullary mass of immature myeloid cells, often in patients with myeloid leukemia. Myeloid sarcoma of the breast is extremely uncommon, and bilateral involvement is even rarer. Myeloid sarcoma of the breast can mimic primary breast cancer, lymphoma, and other neoplasms. Differentiation between myeloid sarcoma and primary breast malignancy is imperative, as management and treatment are drastically different. We present a case of myeloid sarcoma of both breasts in a 63-year-old female with relapsed acute myelogenous leukemia (AML), a personal history of ovarian cancer, and a family history of both leukemia and breast cancer. This report highlights the need for high clinical, radiological, and pathological suspicion to diagnose myeloid sarcoma of the breast.

Teaching NeuroImage: Intradural ALK-Positive Histiocytosis With Involvement of Nerve Roots and Spinal Dura.

A 3-year-old boy presented with progressive lower extremity weakness and difficulty walking for 2 months. Neurologic examination found the muscle strength of his left lower limb was grade 3 and tendon reflex was weakened. MRI demonstrated an intradural extramedullary mass between T10-11 with multiple and diffuse enhancement of thickened nerve roots and spinal dura (Figure). Juvenile xanthogranuloma was suspected as positive staining for CD68 and negative for S100 and CD1a, while immunoreactivity for ALK did not support it. The genetic test confirmed the diagnosis of ALK-positive histiocytosis with KIF5B-ALK fusion, which is a relatively new subtype of histiocytic disease.1,2

Cervical intradural extramedullary cavernous malformation as a rare cause of subarachnoid hemorrhage without spinal dysfunction: illustrative case

BACKGROUNDIntradural extramedullary cavernoma is a very rare lesion of the spinal cord, especially of the cervical spine. Its clinical presentation can vary with symptoms of sensory or motor deficits and even with symptoms of subarachnoid hemorrhage (SAH).OBSERVATIONSThe authors present a case of a 45-year-old man with SAH with prolonged neck pain and increasing headache confirmed by lumbar puncture. Head computed tomography revealed only discrete blood deposits in the right frontal and biparietal lobes. The finding of pan-cerebral angiography was negative for the cause of bleeding. Spinal magnetic resonance imaging revealed an intradural extramedullary mass lesion at cervical level C5–6. The finding of subsequent cervical angiography was negative. The diagnosis of a cavernous malformation was confirmed histopathologically after surgery. The cavernoma was completely removed, and full recovery of the initial symptoms was achieved.LESSONSSpinal lesions should be considered in the diagnostic work-up for SAH with excluded origin of bleeding in cranial neuroimaging. An intradural extramedullary cavernous malformation is an extremely rare entity in the differential diagnosis of SAH, and surgical resection is the treatment of choice to prevent further bleeding and neurological deficits.

Spinal medulloblastoma in pregnancy: A pandora of challenges for the anesthesiologist

Tumors of the spinal column, meninges, nerve roots, or the cord parenchyma itself may compress and damage the spinal cord resulting in neural deficits. The presence of spinal cord tumor itself in pregnancy is a rare occurrence. Rapidly progressive neurological involvement due to compression should be considered for immediate decompression. The specific positioning for surgery and inaccessibility to the airway during surgery makes all spinal surgeries technically challenging for the anesthesiologist. The presence of pregnancy along with this disease further complicates patient management. We present the case of a 24-week-old pregnant female who reported to emergency with decreased sensation in her lower limbs and urinary retention. She was operated previously for cranial medulloblastoma and was on regular follow-up. Magnetic resonance imaging spine revealed multiple intradural extramedullary masses at the D3-4, D8-9, and D10-D11 vertebral levels. Considering her pregnancy and increased risk of surgery during this condition, only the mass causing her symptoms was decided to be operated upon (D10-11). The surgery was done in the prone position, during which, fetal well-being was insured by the obstetric team using transabdominal ultrasonography. Careful padding of pressure points was done; frames on the Allen table were placed in a manner to avoid any pressure on the abdomen during the surgery. The surgery was uneventful, and the patient was successfully reversed and extubated. Fetal heart rate was monitored perioperatively at regular intervals, and ultrasonographic assessment was done by the obstetric team once the patient was extubated. This case highlights the requirement of training the professionals for tackling nonobstetric surgery in a pregnant patient. The increased awareness and education in society regarding perioperative care issues will bring forth new challenges and thus we need to be more prepared for handling such cases.

Clinically aggressive pediatric spinal ependymoma with novel MYC amplification demonstrates molecular and histopathologic similarity to newly described MYCN-amplified spinal ependymomas

Primary spinal cord tumors contribute to ≤ 10% of central nervous system tumors in individuals of pediatric or adolescent age. Among intramedullary tumors, spinal ependymomas make up ~ 30% of this rare tumor population. A twelve-year-old male presented with an intradural, extramedullary mass occupying the dorsal spinal canal from C6 through T2. Gross total resection and histopathology revealed a World Health Organization (WHO) grade 2 ependymoma. He recurred eleven months later with extension from C2 through T1-T2. Subtotal resection was achieved followed by focal proton beam irradiation and chemotherapy. Histopathology was consistent with WHO grade 3 ependymoma. Molecular profiling of the primary and recurrent tumors revealed a novel amplification of the MYC (8q24) gene, which was confirmed by fluorescence in situ hybridization studies. Although MYC amplification in spinal ependymoma is exceedingly rare, a newly described classification of spinal ependymoma harboring MYCN (2p24) amplification (SP-MYCN) has been defined by DNA methylation-array based profiling. These individuals typically present with a malignant progression and dismal outcomes, contrary to the universally excellent survival outcomes seen in other spinal ependymomas. DNA methylation array-based classification confidently classified this tumor as SP-MYCN ependymoma. Notably, among the cohort of 52 tumors comprising the SP-MYCN methylation class, none harbor MYC amplification, highlighting the rarity of this genomic amplification in spinal ependymoma. A literature review comparing our individual to reported SP-MYCN tumors (n = 26) revealed similarities in clinical, histopathologic, and molecular features. Thus, we provide evidence from a single case to support the inclusion of MYC amplified spinal ependymoma within the molecular subgroup of SP-MYCN.

Clinicopathological analysis of myeloid sarcoma with megakaryocytic differentiation

Myeloid sarcoma (MS) is defined as a tumour mass consisting of myeloid blasts that occurs at an anatomical site other than bone marrow. MS with megakaryocytic differentiation (MSmgk) is extremely rare and its clinicopathological features have not been well described. We reviewed 11 cases in 11 patients of extramedullary mass-forming malignant tumours composed of immature non-lymphoid haematopoietic cells expressing CD41 with or without concurrent bone marrow lesions. The patients consisted of seven men and four women (1.75:1 male-to-female ratio). The mean and median ages at diagnosis were 50 and 62 years, respectively, ranging from 2 to 78 years. Extramedullary mass lesions were solitary in three cases (27%) and multiple in eight cases (73%). Tumour locations were lymph nodes (6 cases), subcutaneous tissue (3 cases), intramuscular (1 case), and bone (1 case). Seven of the 11 patients (64%) had a history of myelodysplastic syndrome (MDS) or myeloproliferative neoplasm (MPN). Three patients (27%) developed MS during remissions of acute myelogenous leukaemia, and one patient had a recurrence of MS at other sites. Follow-up data were available for four cases. Tumour cells were positive for CD41, CD33, CD34, MPO, and CD68 in 11 (100%), three (27%), seven (64%), four (36%), and seven (64%) cases, respectively. Cytogenetic analysis was successfully performed in two cases. Complex but inconsistent abnormalities were evident. When compared with cases of MS without megakaryocytic differentiation, the survival of MSmgk was significantly shorter (p=0.0033). Compared to MS without megakaryocytic differentiation, MSmgk is more likely to follow MDS/MPN, to involve multiple sites, and to be associated with poorer outcomes. More detailed studies, including genomic or gene expression analyses, could confirm the characteristics of MSmgk.

Intradural Extramedullary Lesions in Cervical Spine in Neurofibromatosis

Introduction: Neurofibromatosis (NF) is isolated into three diseases: NF type 1, type 2, and schwannoma. NF type 2 could be a disorder that's found roughly in 1/25,000–33,000 births with a mutation in gene 22q11.2, and it is passed through eras in an autosomal dominant fashion. Diagnosis is made with both clinical and radiological features. A few clinical features have been characterized in conclusion counting Manchester criteria. There is a scarce number of NF type 2 patients diagnosed with cervical lesions which are 25 in number. We report a case of an intradural extramedullary cervical lesion in a patient later diagnosed with NF type 2.
 Case Report: A 30-year-old male presenting with gradual onset and progressive course of spastic quadriparesis of six months’ duration was admitted through the emergency unit. MRI spine showed intradural extramedullary masses in the right side of C4 and left side of C6. The patient underwent cervical intradural excision of two masses under general anesthesia with neuromonitoring. The tumor was sent for histopathology and reported as NF type 2.
 Conclusion: NF is a common entity, but the diagnosis of cervical mass is judicious to avoid any complication in neurological function. It further needs a multidisciplinary approach and screening modalities.

Atypical Targetoid Lesions as the Presenting Sign of Myeloid Sarcoma

Myeloid sarcoma (MS), also known as chloroma or granulocytic sarcoma, is a rare extramedullary tumor mass that commonly occurs in acute myeloid leukemia (AML). MS may occur before other clinical signs of AML, or alongside other known AML symptoms. MS can also appear in isolation, which makes diagnosis challenging. Clinical knowledge about a typical presentation of myeloid sarcoma is limited, given the heterogeneity in the location and size of MS lesions. We describe a patient with a history of AML who developed an eruption of atypical targetoid purpuric lesions. Diagnostic workup, including laboratory and histologic specimen from the skin were consistent with MS. MS should be suspected in a patient with a history of myeloproliferative disorder who develops atypical targetoid purpuric papules.

Thoracic calcified disc sequestration presenting as a spinal extramedullary intradural mass

Thoracic calcified disc sequestration presenting as a spinal extramedullary intradural mass

Evolution of a refractory prolactin-secreting pituitary adenoma into a pituitary carcinoma: report of a challenging case and literature review

BackgroundPituitary carcinomas (PCs), defined as distant metastases of pituitary neoplasms, are very rare malignancies. Because the clinical presentation of PCs is variable, early diagnosis and management remain challenging. PCs are always refractory to comprehensive treatments, and patients with PCs have extremely poor prognoses.Case presentationWe describe one case of a prolactin-secreting pituitary adenoma (PA) refractory to conventional therapy that evolved into a PC with intraspinal metastasis. A 34-year-old female was diagnosed with an invasive prolactin-secreting PA in 2009 and was unresponsive to medical treatment with bromocriptine. The tumor was gross totally removed via transsphenoidal surgery (TSS). However, the patient experienced multiple tumor recurrences or regrowth despite comprehensive treatments, including medical therapy, two gamma knife radiosurgeries (GKSs), and four frontal craniotomies. In 2016, she was found to have an intradural extramedullary mass at the level of the fourth lumbar vertebra. The intraspinal lesion was completely resected and was confirmed as a metastatic PC based on histomorphology and immunohistochemical staining. The literature on the diagnosis, molecular pathogenesis, treatment, and prognosis of patients with prolactin-secreting PCs was reviewed.ConclusionPCs are very rare neoplasms with variable clinical features and poor prognosis. Most PCs usually arise from aggressive PAs refractory to conventional therapy. There is no reliable marker to identify aggressive PAs with a risk for progression to PCs; thus, it is difficult to diagnose these PCs early until the presence of metastatic lesions. It is still very challenging to manage patients with PCs due to a lack of standardized protocols for diagnosis and treatment. Establishing molecular biomarkers and the pathobiology of PCs could help in the early identification of aggressive PAs most likely to evolve into PCs.