Spinal Hamartoma Research Articles

Clinical Features and Surgical Outcomes of Spinal Hamartomas: Surgical Treatment of 8 Patients

ObjectiveRarely do spinal hamartomas receive attention in the literature, and the majority of previous studies consist of case reports. The purpose of this report, then, is to provide a more accurate diagnosis and treatment of spinal hamartomas by presenting the clinical and surgical outcomes of 8 cases. MethodsThe researchers conducted a retrospective analysis of the data for eight individuals with spinal hamartomas. The eight patients had undergone both preoperative and postoperative magnetic resonance imaging (MRI) and went through surgery. Radiological and pathological criteria were applied to diagnose the spinal hamartomas. The Modified McCormick classification system was used to evaluate neurological function in these patients. ResultsAmong the eight patients, six were male and two were female, with an average age of 37.4 years. The typical duration of symptoms was 14.5 months. During surgery, all lesions were well-defined and located in the bone marrow. Gross total resection (GTR) was achieved in 6 cases, and the anatomical plane was clearly delineated, and subtotal resection (STR) was achieved in 2 cases. None of the patients took postoperative radiotherapy. 14.5 months was the average follow-up period, during this time, MRI did not observe the recurrence or regeneration of residual tumors. The McCormick grade was improved in seven patients and remained stable in one patient. ConclusionsSpinal hamartomas are benign, but they are clinically progressive lesions. Pathology is the basis of accurate diagnosis, and complete preoperative evaluation is crucial for providing suitable treatment and prognosis.

Sporadic Spinal Hamartoma in Adults: A Rare Pathologic Entity

Sporadic spinal hamartomas in adults are extremely rare tumor-like lesions, without defined guidelines of treatment. The aim of this study is to investigate the peculiar features of this pathologic entity to support a more accurate diagnosis and management. A comprehensive and detailed literature review of sporadic spinal hamartomas in adults, including a personal case, was performed. Demographic (sex and age), clinical (presenting symptoms), radiologic and pathologic (size, localization, dural and spinal cord relationship of lesion) features, as well as treatment (time to surgery, extent of resection, perioperative and postoperative complications) and outcome (clinical) data were analyzed. Seven studies, including 7 patients, were eligible for the review. A personal case was also added. Sporadic adult spinal hamartomas equally affect males and females, in a range of age from 18 to 75 years; sensory radicular deficits were detected in all patients at clinical onset; thoracic segment (4/8) of the spine and intradural compartment (6/8) were most involved. All patients underwent surgery and just one experimented postoperative complication; gross total resection was achieved in only 3 patients, mainly in an extradural localization; clinical improvement at last follow-up was reported in all but 1 patient. Spinal hamartomas in adults may sometimes account for progressive worsening of neurologic symptoms and lead to potentially irreversible neurologic deficit; therefore, prompt and adequate diagnosis and treatment are mandatory. Surgical resection represents the only curative treatment and is indicated for symptomatic lesions to achieve neurologic symptoms restoration or arrest/prevent their progressive deterioration.

Adult Spinal Hamartoma Involving Conus Medullaris: Brief Review About Associated Congenital Abnormalities and Surgical Outcome

Background: Spinal hamartoma is an extremely rare lesion for symptomatic spinal cord compression in adult population. Without any association of spinal dysraphism and neurofibromatosis, only 3 cases have been reported in the literature. Case description: A 40-year-old man presented with lumbago femorica for 6 months. Gradually the symptoms worsened and he developed features of Cauda equina syndrome. Magnetic Resonence Imagimg demonstrated a heterogeneously hyperintense intradural extramedullary mass in both T1 and T2WI, involving conus medullaris and cauda equina, opposite to L1. Patient underwent near total resection of lesion through posterior midline approach. Histopathological features were consistent with hamartoma. Patient neurologically improved and follow up MRI at 6 months showed static small nodular tumor residue. Conclusion: To the best of our knowledge, current report is the fourth case of spinal hamartoma in adult male, without any association of spinal dysraphism and neurofibromatosis .This may be the first reported case from Bangladesh.

Case 2: A Midline Lumbosacral Mass

A 39-week-male infant is born to a 35-year-old, gravida 4 now para 4 mother whose pregnancy is uncomplicated. He is born by spontaneous vaginal delivery at an outside hospital, and no resuscitation is required. Apgar scores are 9 and 9 at 1 and 5 minutes, respectively. Birth weight, occipital circumference, and length are all in the 90th percentile. On initial examination, there is a 4 × 5-cm midline mass at the level of the lumbosacral spine (Figure 1). The mass is well circumscribed, has a smooth surface except for the protruding ‘tail,” is covered by normal skin, is not pulsatile, and slips under the palpating finger. Protruding from the mass is a 1-cm-long skin tag. He moves all 4 extremities spontaneously. Tone in the legs is normal, and deep tendon reflexes in the lower extremities are 2+. He withdraws all extremities to pain. The findings of the rest of the neurologic examination, including cranial nerves and motor examination, are normal. His anus is patent with normal sphincter tone and a normal anal reflex (anal wink is present). He has a sexual maturity rating of 1 for male genitalia, and both testes are palpable. The remainder of the examination findings are unremarkable. He is transferred to a neonatal intensive care unit for further evaluation and management. He has voided and stooled before transport. An additional bedside test gives a clue to the diagnosis. Figure 1. Lumbosacral mass with appendage. ### Diagnosis The differential diagnosis of a midline lumbosacral mass includes sacrococcygeal teratoma with or without the Currarino triad, hemangioma, spinal hamartoma, congenital ependymoma, caudal regression syndrome, and occult spinal dysraphism (OSD). Our patient lacks obvious anorectal …

Midline Spinal Cord Hamartomas—A Report of Six Cases

Midline Spinal Cord Hamartomas are relatively rare and asymptomatic. These lesions may present with a tethered cord and a normal overlying skin, a skin dimple, a dermal sinus tract or dense collagen. Most patients have few or no neurological symptoms related to them. Microscopically they are composed of mature and well-differentiated ectodermal and mesodermal elements, meningothelial cells, calcification, and even urothelium. We report MRI and histologic features of midline spinal cord hamartomas in six cases.

A spinal hamartoma in an aged woman

Spinal harmatoma is an extremely rare tumor, and only 16 cases have been reported previously. We report on a 73-year-old woman with low back pain because of spinal hamartoma. To our knowledge, the current report is the first published case of a spinal hamartoma without other systemic diseases in the aged.

Spinal cord hamartoma with pseudopancreatic cyst

A 2-year-old boy presented with weakness of both lower limbs with bladder and bowel involvement and history of frequent falls. Magnetic resonance imaging of the spine revealed a T2 altered signal intensity enhancing mass lesion seen in the spinal epidural space extending from sixth cervical to fourth thoracic vertebrae, which was compressing the adjacent spinal cord. Histopathology of the lesion was suggestive of hamartoma. A brief review of the literature including its embryogenesis is discussed here. The child subsequently developed pseudopancreatic cysts possibly because of repeated falls leading to blunt trauma abdomen.

Spinal hamartoma associated with spinal dysraphism

The aims of the study were (1) to review the pathological findings of spinal lipomatous masses associated with congenital spinal dysraphism and (2) to discuss the pathological diagnosis. The pathological records of 47 patients at our institution were reviewed, and three illustrative cases were presented. Spinal tumorous lesions associated with spinal dysraphism have been traditionally described as lipoma since they are composed mostly of fatty tissue. However, they are different from lipomas arising in other part of the body in that they often contain various tissues of ecto- and mesodermal origin. In our study, we detected such heterotopic components in 24 out of 47 cases. Although they are also similar to teratoma, it is generally accepted that they are malformative lesions which lack neoplastic potential. We therefore should diagnose them as hamartoma rather than lipoma or teratoma.

Establishment of a clonal human mesenchymal cell line that retains multilineage differentiation capacity from a spinal hamartoma.

We isolated a single-cell-derived cell line from a spinal hamartoma, a which occurred in a newborn boy and was associated with a rudimentary limb. The maternal cells (HHC-7) differentiated into osteoblasts, chondrocytes, adipocytes, and skeletal muscles when they were cultured in differentiation-inducing media specific to each mesenchymal cell. We isolated a single-cell-derived clonal cell line (Clone K) after transfection with SV40 T antigen. These cells expressed CD73 and CD117, while being negative for expression of CD45. Clone K cells cultured in an osteogenic differentiation medium increased ALP activity and expressed mRNAs for Runx2 and osteocalcin. Treatment with rhBMP-2 induced Clone K cells to differentiate into both osteoblasts and chondrocytes. These cells expressed mRNAs for Sox9 and aggrecan in addition to osteogenic markers. Culture in an adipogenic differentiation medium induced Clone K cells to differentiation into adipocytes, which expressed mRNAs for PPARgamma2 and a2P. Clone K cells cultured in a serum-depleted medium generated desmin-positive cells and expressed MyoD1 mRNA. Clone K cells exhibited numerous alpha-smooth muscle actin-positive cells; however, treatment with rhBMP-2 decreased their number. Clone K cells, transplanted with a carrier containing rhBMP-2 into the muscles of SCID mice, generated ectopic endochondral bone formation. In these tissues, several osteoblasts and chondrocytes expressed SV40 T antigen, indicating their Clone K cell origin. Thus, Clone K cells are useful tools for analyzing the characteristics of human multipotential mesenchymal progenitors.

Spinal hamartomas: a distinct clinical entity.

Congenital spinal hamartomas are defined as tumors of well-differentiated mature elements situated in an abnormal location. In this report, the authors document the clinical and pathological features of spinal hamartomas in 10 patients. Ten patients presented with midline dorsal malformations at birth, initially diagnosed as teratomas or myelomeningoceles. The locations of the masses were variable: two were located in the thoracic region, four at the thoracolumbar junction, two in the lumbar region, one at the lumbosacral junction, and one in the sacral region. The results of the neurological examination were normal in nine patients. All but one mass had intact skin and seven had palpable bone components. Neuroimaging studies revealed widening of the spinal canal, heterotopic bone located dorsally in some patients, and varying degrees of involvement of the intraspinal contents. During surgery, six patients were found to have involvement of the spinal cord or cauda equina. The pathological characteristics of the masses included three or more of the following: bone, cartilage, synovial membrane, urinary tract tissue, cyst wall, yellow or brown fat, and nerves. The well-differentiated cellular elements, which formed mature structures, along with the absence of primitive cellular components and neoplastic characteristics are more consistent with a diagnosis of hamartoma than teratoma. In this series, the authors describe a lesion that is overt on physical examination, yet can have occult spinal canal involvement. Complete neurosurgical evaluation is essential to provide appropriate treatment and prognosis.

Occult dysraphism in adulthood: clinical course and management

We present a series of 23 patients with dysraphic malformations and adult onset of symptoms (4 meningoceles, 19 spinal hamartomas). Mean age at presentation was 39 +/- 21 years (range 23-67 years). Patients were followed up for a mean period of 19 months (range 0.5-68 months). Only patients with progressive neurological disease were operated on (3 meningoceles and 16 spinal hamartomas). The remaining patients were treated conservatively and continue to be observed clinically. Two of three patients operated for meningoceles improved without recurrence of symptoms. Patients with spinal hamartomas could be divided into two groups according to their main symptom: paraparesis (group A, n = 8) or pain (group B, n = 11). Malformations in group B were typically associated with a tethered cord and tended to be more complex than in group A. The majority of patients in group A showed better long-term results than patients in group B, due to their considerably lower rate of recurrence.

Use of Anal Sphincter Electromyography during Operations on the Conus Medullaris and Sacral Nerve Roots

The mechanical activity of the anal sphincter can be translated into electrical activity and recorded on graph paper or an oscilloscope. The activity of the anal sphincter may be extrapolated to activity of the external urethral sphincter because both are striated muscles innervated by the pudendal nerve that arises from S-2, S-3, and S-4. Stimulation of these nerves causes contraction of the sphincter muscles, and a deflection of the recording device occurs. This technique was employed intraoperatively in monitoring operations on the conus medullaris and sacral nerve roots in 10 patients with spinal dysraphism (age range, 3 weeks to 15 years). Their diagnoses were tethered conus, 4; lipomeningocele, 3; spinal hamartoma, 1; syringocele, 1; and sacral arachnoiditis, 1. With general anesthesia, and the patient in the prone position, an electrode-containing anal plug was inserted or two needle electrodes were inserted into the anal sphincter muscle. The electrodes were connected to the electromyography recording stylus of the urodynamic bladder diagnostic unit. During the spinal operation, whenever a structure could not be identified clearly, it was stimulated with the disposable electrical stimulator and, if oscillations of the stylus occurred (indicating contraction of the anal sphincter), the structure was preserved. This technique permitted spinal operations in these 10 patients without changes in neurological or urological function.