A case of macrodactyly associated with neurofiborma of the median nerve, a congenital anomaly of the hand, affecting only one(left middle) finger is reported with a review of the literature. Macrodactyly which is also termed local gigantism, megalodactylism, megalodactylia, or macrodactylism in other literature, is a rare congenital malformation characterized by overgrowth of one or more fingers of hand. Macrodactyly associated with neurofibroma of the median nerve is especially rare. For this reason the following case is presented together with a review of the literature.
Adolescent ; Angiography ; Female ; Fingers/abnormalities* ; Fingers/radiography ; Fingers/surgery ; Human ; Korea ; Median Nerve*/surgery ; Neurofibroma/complications* ; Neurofibroma/pathology ; Neurofibroma/surgery ; Peripheral Nervous System Neoplasms/complications* ; Peripheral Nervous System Neoplasms/pathology ; Peripheral Nervous System Neoplasms/surgery ; Radial Nerve/surgery

Schwannomatosis or neurilemmomatosis has been used to describe patients with multiple nonvestibular schwannomas with no other stigmata of neurofibromatosis type-2 (NF-2). In our case, schwannomatosis, multiple schwannomas were present in a 21-yr-old woman with no stigmata or family history of NF-1 or NF-2. She had no evidence of vestibular schwannoma or other intracranial tumors. Multiple peripheral tumors were found in the carotid space of the neck, and soft tissue of posterior shoulder, lower back, ankle and middle mediastinum. All of those tumors were completely limited to the right side of the body. All surgically removed tumor specimens in this patient proved to be schwannomas.
Treatment Outcome ; Peripheral Nervous System Neoplasms/*diagnosis/*surgery ; Neurilemmoma/*diagnosis/*surgery ; Humans ; Female ; Adult

OBJECTIVETo study the use of a nerve ''bypass'' graft as a possible alternative to neurolysis or segmental resection with interposition grafting in the treatment of neuroma-in-continuity.

METHODSA sciatic nerve crush injury model was established in the Sprague-Dawley rat by compression with a straight hemostatic forceps. Epineurial windows were created proximal and distal to the injury site. An 8-mm segment of radial nerve was harvested and coaptated to the sciatic nerve at the epineurial window sites proximal and distal to the compressed segment (bypass group). A sciatic nerve crush injury without bypass served as a control. Nerve conduction studies were performed over an 8-week period. Sciatic nerves were then harvested and studied under transmission electron microscopy. Myelinated axon counts were obtained.

RESULTSNerve conduction velocity was significantly faster in the bypass group than in the control group at 8 weeks (63.57 m/s+/-5.83 m/s vs. 54.88 m/s+/-4.79 m/s, P<0.01). Myelinated axon counts in distal segments were found more in the experimental sciatic nerve than in the control sciatic nerve. Significant axonal growth was noted in the bypass nerve segment itself.

CONCLUSIONNerve bypass may serve to augment peripheral axonal growth while avoiding further loss of the native nerve.

Animals ; Male ; Neural Conduction ; Neuroma ; surgery ; Peripheral Nerves ; transplantation ; ultrastructure ; Peripheral Nervous System Neoplasms ; surgery ; Rats ; Rats, Sprague-Dawley

The authors report on a case of primary malignant melanoma of the 7th cervical spinal nerve root in a 45-year-old woman. Neuro-radiological features of this extra-dural mass were suggestive of a nerve sheath tumor. The lesion underwent total gross resection through the anterolateral approach. The patient's postoperative course was uneventful. Histopathological investigation confirmed malignant melanoma. There was no evidence of tumor recurrence or other melanotic lesions on regular follow-up examinations until the postoperative eighth month. When treating a common, benign-looking lesion of the cervical spinal nerve root, surgeons should be aware of the potential to encounter such a malignant tumor.
Cervical Vertebrae ; Female ; Human ; Melanoma/*pathology/surgery ; Middle Aged ; Nerve Sheath Tumors/*pathology/surgery ; Peripheral Nervous System Neoplasms/*pathology/surgery ; Spinal Nerve Roots/*pathology

Traumatic neuroma is a well-known disorder that occurs after trauma or surgery involving the peripheral nerve and develops from a nonneoplastic proliferation of the proximal end of a severed, partially transected, or injured nerve. We present a case of traumatic neuroma around the celiac trunk after gastrectomy in a 56-year-old man, which was confirmed by pathology. CT demonstrated the presence of a lobulated, homogeneous, hypoattenuating mass around the celiac trunk, mimicking a nodal metastasis.
Celiac Plexus/*pathology/surgery ; Diagnosis, Differential ; Gastrectomy ; Gastric Bypass ; Humans ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoplasms, Post-Traumatic/*diagnosis/surgery ; Neuroma/*diagnosis/surgery ; Peripheral Nervous System Neoplasms/*diagnosis/surgery ; Positron-Emission Tomography ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to investigate the prognostic significance of International Neuroblastoma Pathology Classification (INPC).

METHODSOne hundred and thirty-five cases of peripheral neuroblastic tumors encountered in Shanghai Children's Medical Center were enrolled into the study. All the cases were classified according to INPC and International Neuroblastoma Staging System (INSS). The follow-up data were analyzed.

RESULTSThe consensus diagnoses of the 135 cases were as follows: 80 cases (59.2%) of neuroblastoma, 24 cases (17.8%) of ganglioneuroblastoma, intermixed, 17 cases (12.6%) of ganglioneuroma and 14 cases (10.4%) of ganglioneuroblastoma, nodular. The cases were subdivided into 2 subgroups: favorable histology (number = 90, 66.7%) and unfavorable histology (number = 45, 33.3%). According to INSS, the number of cases in stages I, II, III and IV was 22 (16.3%), 24 (17.8%), 34 (25.2%) and 55 (40.7%), respectively. The survival of peripheral neuroblastic tumors correlated with histologic diagnosis, INPC and INSS (P < 0.05).

CONCLUSIONDiagnostic categorization of peripheral neuroblastic tumors according to INPC is of prognostic value.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; pathology ; surgery ; Ganglioneuroma ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Neuroblastoma ; classification ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; classification ; pathology ; surgery ; Retrospective Studies ; Survival Rate

Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; pathology ; Humans ; Median Nerve ; pathology ; Median Neuropathy ; metabolism ; pathology ; surgery ; Melanoma ; pathology ; Middle Aged ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

OBJECTIVETo evaluate the erectile and ejaculatory function of sacral tumor patients after sacral nerve root resection and investigate the relationship of erectile and ejaculatory dysfunction (EED) with the level of sacral nerve injury.

METHODSThis retrospective study included 47 male patients aged 16 to 63 (32.6 +/- 6.8) years treated by sacral tumor resection between January 2008 and August 2013. According to the levels of the sacral nerve roots spared in surgery, the patients were divided into four groups: bilateral S1-S3 (n=16), unilateral S1-S3 (n=21), unilateral S1-S2 (n=6), and unilateral S1 (n=4). The patients were followed up for 12 to 41 (27.2 +/- 10.9) months by questionnaire investigation, clinic review, and telephone calls about their erectile and ejaculatory function at 3, 6 and 12 months after surgery and in August 2013.

RESULTSIn the bilateral S1-S3 group, the incidence rates of EED were 31.25% (5/16), 25% (4/16), and 12.5% (2/16) at 3, 6, and 12 months respectively after surgery, with recovery of erectile and ejaculatory function in August 2013. The incidence rates of EED in the unilateral S1-S3 group were 85.71% (18/21), 71.43% (15/21), 52.38% (11/21), and 42.86% (9/21) at 3, 6 and 12 months and in August 2013, respectively; those in the unilateral S1-S2 group were 100% (6/6), 83.33% (5/6), 83.33% (5/6), and 66.67% (4/6) at the four time points; and those in the unilateral S1 group were all 100% (4/4). No statistically significant differences were found in the incidence rate of EED among the patients of different ages or tumor types (P > 0.05).

CONCLUSIONThe incidence of postoperative EED in male patients treated by sacral tumor resection is closely related to the mode of operation. Sparing the S3 nerve root at least unilaterally in sacral tumor resection is essential for protecting the erectile and ejaculatory function of the patient.

Adolescent ; Adult ; Ejaculation ; physiology ; Erectile Dysfunction ; epidemiology ; etiology ; Female ; Humans ; Incidence ; Male ; Middle Aged ; Organ Sparing Treatments ; Peripheral Nervous System Neoplasms ; surgery ; Postoperative Complications ; epidemiology ; Postoperative Period ; Retrospective Studies ; Sacrum ; Spinal Nerve Roots ; injuries ; surgery ; Surveys and Questionnaires ; Young Adult

The clinical effect of laparoscopic rectal cancer curative excision with pelvic autonomic nerve preservation (PANP) was investigated. This study evaluated the frequency of urinary and sexual dysfunction of 149 male patients with middle and low rectal cancer who underwent laparoscopic or open total mesorectal excision with pelvic autonomic nerve preservation (PANP) from March 2011 to March 2013. Eighty-four patients were subjected to laparoscopic surgery, and 65 to open surgery respectively. The patients were followed up for 12 months, interviewed, and administered a standardized questionnaire about postoperative functional outcomes and quality of life. In the laparoscopic group, 13 patients (18.37%) presented transitory postoperative urinary dysfunction, and were medically treated. So did 12 patients (21.82%) in open group. Sexual desire was maintained by 52.86%, un-ability to engage in intercourse by 47.15%, and un-ability to achieve orgasm and ejaculation by 34.29% of the patients in the laparoscopic group. Sexual desire was maintained by 56.36%, un-ability to engage in intercourse by 43.63%, and un-ability to achieve orgasm and ejaculation by 33.73% of the patients in the open group. No significant differences in urinary and sexual dysfunction between the laparoscopic and open rectal resection groups were observed (P>0.05). It was concluded that laparoscopic rectal cancer radical excision with PANP did not aggravate or improve sexual and urinary dysfunction.
Adult ; Autonomic Nervous System ; injuries ; Humans ; Laparoscopy ; adverse effects ; Male ; Middle Aged ; Peripheral Nerve Injuries ; etiology ; prevention & control ; Postoperative Complications ; Rectal Neoplasms ; surgery ; Sexual Dysfunction, Physiological ; etiology ; Urologic Diseases ; etiology

OBJECTIVETo study the clinicopathologic features of lipomatosis of nerve (NLS).

METHODSThe clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.

RESULTSThere were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.

CONCLUSIONSNLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; CD56 Antigen ; metabolism ; Carpal Tunnel Syndrome ; complications ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; innervation ; Female ; Hand Deformities, Congenital ; complications ; pathology ; Humans ; Lipoma ; pathology ; Lipomatosis ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Median Nerve ; metabolism ; pathology ; Nerve Sheath Neoplasms ; pathology ; Neurofibroma ; pathology ; Neurofilament Proteins ; metabolism ; Neuroma ; pathology ; Peripheral Nervous System Diseases ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; pathology ; Retrospective Studies ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult