Chronic idiopathic myelofibrosis presenting as cauda equina compression due to extramedullary hematopoiesis: a case report

Extramedullary hematopoiesis (EMH) is occasionally reported in idiopathic myelofibrosis and is generally found in the liver, spleen, and lymph nodes several years after diagnosis. Myelofibrosis presenting as spinal cord compression, resulting from EMH tissue is very rare. A 39-yr-old man presented with back pain, subjective weakness and numbness in both legs. Sagittal magnetic resonance imaging showed multiple anterior epidural mass extending from L4 to S1 with compression of cauda equina and nerve root. The patient underwent gross total removal of the mass via L4, 5, and S1 laminectomy. Histological analysis showed islands of myelopoietic cells surrounded by fatty tissue, consistent with EMH, and bone marrow biopsy performed after surgery revealed hypercellular marrow and megakaryocytic hyperplasia and focal fibrosis. The final diagnosis was chronic idiopathic myelofibrosis leading to EMH in the lumbar spinal canal. Since there were no abnormal hematological findings except mild myelofibrosis, additional treatment such as radiothepary was not administered postoperatively for fear of radiotoxicity. On 6 month follow- up examination, the patient remained clinically stable without recurrence. This is the first case of chronic idiopathic myelofibrosis due to EMH tissue in the lumbar spinal canal in Korea.     

November 2000: 13 year old girl with back pain and leg weakness

A 13 year-old girl presented with back pain and recurrent falls of one year, with more recent loss of ambulation and bladder control. Examination showed spasticity and a sensory level bilaterally at T8. CT and MRI scans showed an epidural soft tissue mass with spinal cord compression and destruction of the pedicle, transverse process and other portions of a mid-thoracic vertebral body. Histologic examination of the gross total resection showed a pigmented villonodular synovitis (PVNS). PVNS is most common in the knee and only 26 cases have been reported in the spine. Although vertebral bodies are rarely involved, it is important to include PVNS in the differential diagnosis of spinal lesions because of its tendency to recur locally if not totally resected.     

Bronchogenic cyst of the conus medullaris with spinal cord tethering: a case report and review of the literature

Bronchogenic cysts (BCs) are congenital malformations that originate from remnants of the primitive foregut. Intraspinal BCs, especially those of the conus medullaris are rare with only one case reported until now. To date, a bronchogenic cyst with spinal cord tethering has not been previously reported. We reviewed the clinical course of a 44-year-old woman, who presented with low back pain and leg weaknesss as well as sphincter disturbance. Magnetic resonance imaging showed an intradural oval mass located at the conus medullaris. A tethered cord was also observed, as well as a dermal sinus tract. The mass was totally removed after an L3-L4 laminectomy without detethering during operation. Pathologic examination confirmed the diagnosis of bronchogenic cyst. By six months after treatment, the patient had experienced nearly complete recovery. The review of literature indicated that detethering was performed in most reported cases of neurenteric cysts with spinal cord tethering, and one of six patients was diagnosed with a postoperative recurrence. The co-existence of bronchogenic cyst and a tethered spinal cord would imply associated developmental errors in embryogenesis. It is worth noting that whether detethering is necessary after the cyst removal.     

Diffuse cerebrospinal gliomatosis with extensive leptomeningeal spread

A case of diffuse cerebrospinal gliomatosis with extensive leptomeningeal spread is presented. The patient, an 18-year-old girl, was admitted due to progressive weakness and paresthesia of both legs, following rapid neuropsychiatric deterioration. An initial magnetic resonance imaging (MRI) study of the T-spine showed diffuse high signal intensities from T9 to T12 spinal cords on a T2 sagittal image and diffuse cord bulging at T1WI. This suggested an inflammatory lesion such as tuberculosis or fungal meningoencephalitis. A limited autopsy was performed. A microscopic examination revealed multifocal GFAP-positive astrocytic proliferations that were low grade astrocytoma in the cerebral leptomeninges, parietal, occipital and temporal lobes and anaplastic astrocytoma in the spinal cord and spinal leptomeninges. The high proliferative indices of the spinal lesion and aneuploidy correspond to a diagnosis of malignant astrocytoma and a rapid fatal clinical course.     

Spinal epidural granulocytic sarcoma preceding acute myelogenous leukemia.

A rare case of spinal epidural granulocytic sarcoma (GS) preceding acute myelogenous leukemia is described. A 10-year-old boy presented with lower leg weakness. The initial diagnosis was a histiocytic lymphoma, and he was treated accordingly. No evidence of bone marrow involvement was found at that time. The correct diagnosis of epidural GS was made possible in retrospect by using immunoperoxidase staining for lysozyme fourteen months later when the patient showed the full-blown features of leukemia. This rare tumor should be considered in the differential diagnosis of an epidural mass with cord compression in patients with or even without acute leukemia, because early diagnosis followed by appropriate combined chemotherapy and radiation may obviate surgical intervention and eventually prevent leukemic transformation.     

Fine-needle aspiration biopsy diagnosis of intrathoracic extramedullary hematopoiesis presenting as a posterior mediastinal tumor in a patient with sickle-cell disease: Case report

Extramedullary hematopoiesis (EMH) is a rare cause of an intrathoracic mass. Fine-needle aspiration biopsy (FNAB) has been only occasionally documented as a useful tool in diagnosing EMH tumor. We report a case of posterior mediastinal extramedullary hematopoietic mass in an 80-yr-old man with sickle-cell anemia. The mass was revealed incidentally on chest X-ray. The definitive diagnosis of this mass lesion was achieved by FNAB. The cytologic smears showed hematopoietic elements with erythroid hyperplasia. A correct cytologic diagnosis can thus help to avoid unnecessary surgical intervention, particularly in an asymptomatic patient.     

Endoscopic ultrasound guided fine‐needle aspiration of a splenic hemangioma with extramedullary hematopoiesis

Extramedullary hematopoiesis (EMH) is the production of mature blood elements outside of the bone marrow and can occur as a compensatory result of a marrow replacing process or from marrow space occupying lesions such as tumor or marrow fibrosis. EMH can also be induced by factors elicited by neoplasms, such as vascular endothelial growth factor (VEGF). Usually, EMH is a diffuse process most commonly observed in lymph nodes, liver, and spleen. Rarely, EMH can form a mass lesion. Although the spleen is a common site for diffuse EMH, it is a rare location for a mass forming EMH. Hemangiomas are the most common benign tumors of the spleen. A case of a discrete, 8 cm lesion was noted incidentally on CT scan in a 59‐year‐old man with no significant past medical history. Endoscopic ultrasound guided fine‐needle aspiration (EUS FNA) biopsy was performed and cytologic examination revealed trilinear hematopoiesis, with the most distinctive elements being megakaryocytes and erythroid precursors. A diagnosis of EMH was made. On resection, the mass was a hemangioma with EMH. EUS guided FNA is a useful tool for diagnosing splenic masses. Awareness of EMH, both as a mass forming lesion and a feature associated with benign and malignant vascular lesions is important, especially in patients with hematologic malignancies or marrow replacing processes. Diagn. Cytopathol. 2013;41:1086–1090. © 2011 Wiley Periodicals, Inc.     

A progressive multifocal neurological syndrome in a 42-year-old woman

This is the case of a 42-year-old female who presented with transient dizziness. Her symptoms and signs progressed to include dysarthria, ataxia and cognitive decline over 2 years, such that she was unable to care for herself. She died 4 years after first presentation without a diagnosis. Investigations revealed a normochromic normocytic anaemia. Cerebrospinal fluid was normal. Serial computed tomography brain showed a wedge-shaped frontal infarct but no progressive changes. Examination at autopsy showed discoloration of the gray and white matter of the brain and spinal cord.Microscopy of leptomeningeal and parenchymal vessels showed they were filled with atypical B lymphocytes confined to the intravascular space with multiple infarcts in the brain, cerebellum and spinal cord. A diagnosis of intravascular B cell lymphoma was made and is discussed.     

Fibrous histiocytoma of orbit: a case report

A fifty year male (K.S.) presented with protrusion (Proptosis) of left eye which was gradually increasing in size for the last one year. The swelling was accompanied by pain for the last fifteen days. Preoperative X-ray & computed tomography showed erosion of frontal sinus & roof of maxillary sinus. Clinical diagnosis of a malignant tumour of eye was made & enucleation of the left eye, along with the mass was done. The specimen was received in the pathology department on 16.9.98. Grossly, encapsulated mass attached with the intact eye ball was received. The mass measured 5x3x2.5 cms. Microscopic examination revealed a benign spindle cell neoplasm and a diagnosis of fibrous histiocytoma (dermatofibroma) was made.     

35-year-old woman with progressive bilateral leg weakness

A 35-year-old woman presented with one month's history of progressive bilateral leg weakness and altered sensation. There had been no pain. She had noted urinary frequency and constipation in the previous two weeks. On examination, the patient had diffuse lower extremity weakness (2-3/5), with a T6 sensory level to pain and temperature sensation. MRI demonstrated a T4-5 intradural mass ventral to the spinal cord, with an enhancing dural tail, consistent with meningioma. At surgery an intradural, extramedullary, firm, black neoplasm was encountered, which invaded the ventral dura and elevated and distorted the spinal cord. The mass was removed, leaving only microscopic invasion of the ventral dura. There was no bone invasion. Serial sections revealed a homogeneous black tumor without necrosis. H&E stained sections showed an occasionally fascicular tumor of melanocytes and small round blue tumor spindle cells with melanin pigmentation and 1-2 mitotic figures per 10 high-powered fields. The nuclei are generally oval-shaped and elongated, with prominent nucleoli. Necrosis, hemorrhage, and nuclear and cellular pleomorphism are not present and mitotic figures are rare. Immunohistochemical staining was positive for S-100 and HMB-45. MIB-1 labeling averaged 1-2%. A diagnosis of primary meningeal melanocytic tumor was made. Primary meningeal melanocytic tumors (PMMTs) are rare; fewer than 100 cases have been described. PMMTs of the CNS consist of a spectrum of tumors ranging from well-differentiated melanocytoma to its overtly malignant counterpart, melanoma. Intermediate grade melanocytomas (IMGs) are the least common variant, comprising about 10% of PMMTs reported. IGMS occur in the spinal leptomeninges and intracranially in approximately equal proportions. IGMs are more cellular than the well-differentiated variant, with 1-3 mitotic figures per 10 HPFs and MIB-1 labeling of <6%. By contrast, melanomas contain more mitotic figures (3-15 per 10 HPF) and MIB-1 labeling rates up to 15%. Once metastasis, including drop metastasis from pigmented medulloblastomas, have been excluded, the differential includes pigmented meningiomas and schwannomas (solitary or as part of Carney complex), as well as other pigmented CNS tumors such as ependymoma and pineoblastoma and systemic diseases such as lymphoma . . . For primary CNS melanocytic neoplasms, complete tumor resection is preferred, as it leads to cure of well-differentiated and intermediate-grade melanocytomas and most melanomas. Radiotherapy is recommended for incomplete resection of IMGs and melanomas; the recurrence potential of low-grade melanocytomas is less clear and watchful waiting may be employed, since recurrent tumors may be treated surgically prior to radiation. Two months after surgery, the patient had normal sensation and strength. She was given focused radiotherapy to the region of the ventral thecal sac to 40 cGy. At one year following surgery, the patient's neurological examination is normal and she remains free of residual disease by MR examination.     

Craniospinal dissemination of clival chondroid chordoma

Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment.     

Spontaneous resolution of paraparesis because of acute spontaneous thoracolumbar epidural hematoma

Symptomatic spontaneous spinal epidural hematoma(SSEH) is an uncommon cause of cord compression that commonly is considered as an indication for emergent surgical decompression. We aimed to investigate a patient with a SSEH that completely resolved clinically and radiographically, without surgical treatment. The patient presented three days after the sudden onset of back pain, numbness, and weakness. Magnetic Resonance Imaging (MRI) revealed a posterior thoracolumbar epidural hematoma extending from the level of T10 to L2 with significant cord compression. Decompression was recommended but he refused surgery and was managed conservatively. One month later, weakness totally recovered and hematoma was absent on MRI.     

Sertoli-Leydig cell tumor with heterologous element: a case report and a review of the literature

The patient was a 19-year-old female who presented with a chief complaint of progressive pelvic pain. Preoperative ultrasound of the right ovary revealed an ovarian torsion as the cause of the patient’s progressive pain. Laparoscopy confirmed the torsion and revealed a right ovary measuring 10 cm in greatest diameter. Intraoperative incision into the ovary revealed a simple ovarian cystic mass measuring 3.0 x 1.5 x 0.8 cm. A solid component within the cyst was identified. Histological sections of the cystic mass demonstrated mononuclear and hyperchromatic Sertoli cells with a trabecular growth pattern. Clusters of medium-sized epithelioid cells with abundant eosinophilic cytoplasm consistent with Leydig cells were also identified between the trabeculae of Sertoli cells. In addition, focal areas of intestinal type mucinous epithelium were identified embedded within the trabeculae of Sertoli cells. Immunohistochemical studies revealed that the Sertoli cells were positive for calretinin (bright) while the Leydig cells were positive for calretinin (dim), inhibin, CAM5.2 and AE1&3. CEA showed positivity mainly of the intraluminal contents of the mucinous type intestinal epithelium. The patient had an uneventful post-operative course and was disease-free for 3 years.     

Hindlimb stepping movements in complete spinal rats induced by epidural spinal cord stimulation

The locomotor ability of the spinal cord of adult rats deprived of brain control was tested by epidural spinal cord stimulation. The studies were performed on six rats that had a complete spinal cord transection (T7-T9) and epidural electrode implantations 2-3 weeks before testing was initiated. The stimulating epidural electrodes were implanted at the T12-L6 spinal segments. Epidural electrical stimulation of the dorsal surface of the spinal cord at frequencies between 1 and 50 Hz and intensities between 1 and 10 V without any pharmacological facilitation was used. Stimulation at each of the lumbar spinal cord segments elicited some rhythmic activity in the hindlimbs. However, stimulation at most segmental levels usually evoked activity in only one leg and was maintained for short periods of time (< 10s). Bilateral hindlimb locomotor activity was evoked most often with epidural stimulation at 40-50 Hz applied at the L2 segment. A necessary condition for initiation of locomotor activity was providing a specific amount (at least 5%) of body weight support. Therefore, the rat spinal cord isolated from brain control is capable of producing bilateral stepping patterns induced most readily by epidural stimulation applied at the L2 spinal segment. Furthermore, the induced stepping patterns were dependent on sensory feedback associated with weight bearing.     

Importance of anesthesia for the genesis of neurogenic pulmonary edema in spinal cord injury

There are reports describing both provocation and inhibition of neurogenic pulmonary edema by anesthetic drugs. Therefore, we compared the effect of two types of anesthesia on the formation of neurogenic pulmonary edema in rats with balloon-induced acute spinal cord injury. Animals with sham procedure (group 1) were anesthesized by intraperitoneal sodium pentobarbital. In the experimental groups, rats were submitted to acute spinal cord lesion by insufflations of a balloon in the epidural space at T8 for 1 min (group 3 under i.p. sodium pentobarbital and group 2 under i.p. xylazine-ketamine anesthesia). In rats with pentobarbital anesthesia, systolic blood pressure doubled the baseline value during compression, whereas this effect was less pronounced in the ketamine-xylazine group. The pulmonary index (100 x wet lung weight/body weight) was 0.395 (+/-0.018) in sham-operated rats, rose to 0.499 (+/-0.060) in group 2, and was maximum under pentobarbital anesthesia (0.639+/-0.14; p=0.0018). Histologic examination of the spinal cord showed parenchymal ruptures and acute hemorrhage. Comparison of the pulmonary index with histologic slides of lung parenchyma revealed that relevant intra-alveolar edema occurred only for index values above 0.55. On electron microscopy, endothelial alterations, and damage of the alveolar lining cells were found. Our study indicates that neurogenic pulmonary edema caused by spinal cord injury is less pronounced in rats under xylazine-ketamine anesthesia, when compared with pentobarbital.     

Successful Treatment of Enterocutaneous Fistula in a Hemodialysis Patient with Somatostatin

Although cysticercosis is the most common parasitic disease affecting the central nervous system, spinal cysticercosis is rare. A rare form of spinal cysticercosis involving the whole spinal canal is presented. A 45-year-old Korean male had a history of intracranial cysticercosis and showed progressive paraparesis. Spinal magnetic resonance scan showed multiple cysts compressing the spinal cord from C1 to L1. Three different levels (C1-2, T1-3, and T11-L1) required operation. Histopathological examination confirmed cysticercosis. The patient improved markedly after surgery.     

A Case of Extensive Spinal Cysticercosis Involving the Whole Spinal Canal in a Patient with a History of Cerebral Cysticercosis

Although cysticercosis is the most common parasitic disease affecting the central nervous system, spinal cysticercosis is rare. A rare form of spinal cysticercosis involving the whole spinal canal is presented. A 45-year-old Korean male had a history of intracranial cysticercosis and showed progressive paraparesis. Spinal magnetic resonance scan showed multiple cysts compressing the spinal cord from C1 to L1. Three different levels (C1-2, T1-3, and T11-L1) required operation. Histopathological examination confirmed cysticercosis. The patient improved markedly after surgery.     

Eikenella corrodens cervical spinal epidural abscess induced by a fish bone

Cervical spinal epidural abscess, caused by fish bone injury and a secondary infection by Eikenella corrodens which is part of the normal flora, has not been reported. A 72-yr-old man came to the hospital with pain in his posterior neck and both shoulders for 2 months. He also was experiencing weakness on his right side for 3 days. A fish bone had been stuck in his throat for about 2 months. Neurological examination revealed right hemiparesis, hypesthesia on the left extremities and neck stiffness. Laboratory findings showed an elevated ESR/CRP and leukocytosis, and magnetic resonance imaging revealed a retropharyngeal abscess and cervical myelitis. The patient was treated with emergency surgical decompression and antibiotics. A fish bone was removed from the C3-C4 intervertebral disc space. In the culture of chocolate blood agar and 5% sheep blood agar plate, E. corrodens was detected as a causative organism.     

Primary cytodiagnosis of peritoneal extramedullary hematopoiesis

Cytologic examination of peritoneal fluid in a patient with known myelofibrosis and previous splenectomy revealed megakaryocytes along with erythroid and myeloid precursors. These findings were consistent with extramedullary hematopoietic (EMH) implants of the peritoneum. A few similar cases have been occasionally reported in the literature. This case represents an additional example of a primary diagnosis of peritoneal EMH in which therapy was based on the cytologic findings and sequential cytologic observations were made.     

A common complication of myelofibrosis presenting as a rare finding in cerebrospinal fluid cytology

Herein, we present a rare case of intracranial extramedullary hematopoiesis (EMH) diagnosed by cerebrospinal fluid (CSF) cytology and describe the clinical presentation, radiologic, and pathologic findings. A 65 year‐old man with a history of progressing primary myelofibrosis was admitted for headaches and right facial numbness. A brain MRI revealed focal abnormalities that were suspicious for leptomeningeal involvement of acute leukemia. Cytologic examination of CSF demonstrated a hypercellular specimen composed of hematopoietic cells including few blasts, as well as maturing red blood cells and granulocytic cells. The integration of morphologic findings, peripheral blood and bone marrow counts, as well as flow cytometric analysis of CSF and bone marrow, excluded leptomeningeal involvement by leukemic blasts and helped establish the diagnosis of intracranial EMH. Inclusion of EMH in the differential diagnosis of intracranial pathology in patients with known conditions predisposing them to EMH is important because recognizing this rare event has implications for treatment and prognosis.