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

OBJECTIVE: To construct and validate a nomogram for predicting the risk of secondary peripheral neuropathy in patients with advanced lung cancer. METHODS: The sociodemographic and clinical data of 335 patients with advanced lung cancer admitted to Department of Respiratory, the First Affiliated Hospital of Zhejiang University School of Medicine from May 2020 to May 2021 were retrospectively collected. Pearson correlation analysis, univariate and multivariate logistic regression analyses were used to identify the risk factors of secondary peripheral neuropathy in patients with advanced lung cancer. A nomogram was constructed according to the contribution of each risk factor to secondary peripheral neuropathy, and the receiver operating characteristic (ROC) curve, Calibration curve and clinical decision curve were used to evaluate differentiation, calibration, and the clinical utility of the model. The nomogram was further validated with data from 64 patients with advanced lung cancer admitted between June 2021 and August 2021. RESULTS: The incidences of secondary peripheral neuropathy in two series of patients were 34.93% (117/335) and 40.63% (26/64), respectively. The results showed that drinking history ( OR=3.650, 95% CI: 1.523-8.746), comorbid diabetes ( OR=3.753, 95% CI: 1.396-10.086), chemotherapy ( OR=2.887, 95% CI: 1.046-7.970), targeted therapy ( OR=8.671, 95% CI: 4.107-18.306), immunotherapy ( OR=2.603, 95% CI: 1.337-5.065) and abnormal liver and kidney function ( OR=12.409, 95% CI: 4.739-32.489) were independent risk factors for secondary peripheral neuropathy (all P<0.05). A nomogram was constructed based on the above risk factors. The area under the ROC curve (AUC) of the nomogram for predicting the secondary peripheral neuropathy was 0.913 (95% CI: 0.882-0.944); and sensitivity, specificity, positive and negative predictive values were 85.47%, 81.65%, 71.43% and 91.28%, respectively. The Calibration curve and clinical decision curve showed good calibration and clinical utility. External validation results showed that the AUC was 0.764 (95% CI: 0.638-0.869); and sensitivity, specificity, positive and negative predictive values were 79.28%, 85.79%, 73.25% and 85.82%, respectively. CONCLUSIONS Advanced lung cancer patients have a high risk of secondary peripheral neuropathy after anticancer therapy. Drinking history, comorbid diabetes, chemotherapy, targeted therapy, immunotherapy, abnormal liver and kidney function are independent risk factors. The nomogram prediction model constructed in the study is effective and may be used for the risk assessment of secondary peripheral neuropathy in patients with advanced lung cancer.
Humans ; Nomograms ; Retrospective Studies ; Peripheral Nervous System Diseases/etiology* ; Risk Factors ; Lung Neoplasms/complications*

Peripheral neuropathy or amyotropic lateral sclerosis can be associated with renal cell carcinoma. We report a 63-year-old male patient with renal cell carcinoma who developed an atypical, progressive neuropathy after nephrectomy.
Carcinoma, Renal Cell/*complications/pathology ; Humans ; Kidney Neoplasms/*complications/pathology ; Male ; Middle Aged ; Nephrectomy ; Peripheral Nervous System Diseases/*etiology

Among all the adverse reactions of anti-tumor drugs, the incidence of chemotherapy-induced peripheral neuropathy elevated gradually in recent years, but no trustworthy treatment by Chinese and Western medicine for it has been found so far. The related clinical and experimental reports published in the latest 10 years were reviewed in this paper.
Drug Therapy, Combination ; Drug-Related Side Effects and Adverse Reactions ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Integrative Medicine ; Neoplasms ; complications ; drug therapy ; Peripheral Nervous System Diseases ; drug therapy ; etiology

Inflammatory myofibroblastic tumors (IMTs) are benign neoplasms that can occur at different anatomic sites with nonspecific clinical symptoms. A 48-yr-old woman presented with a 2-month history of a relapsed oral ulcer, progressive dyspnea, and a thoracic pain induced by breathing. A tumorous mass was noticed in the right costodiaphragmatic recess on chest computed tomography and magnetic resonance imaging, and the patient underwent a right costotransversectomy with excision of the tumor, which originated from the 12th intercostal nerve. Histology and immunohistochemistry showed that the tumor was an IMT of the intercostal nerve. The patient's postoperative course was not favorable; dyspnea persisted after surgery, and a progressive pulmonary compromise developed. The cause of the respiratory failure was found to be bronchiolitis obliterans, which in this case proved to be a fatal complication of paraneoplastic pemphigus associated with an IMT. This case of IMT of the spinal nerve in the paravertebral region is unique in terms of its location and presentation in combination with paraneoplastic pemphigus, which is rare. A brief review of the heterogeneous theories concerning the pathogenesis, clinicopathological features, and differential diagnosis of this disease entity is presented.
Diagnosis, Differential ; Fatal Outcome ; Female ; Granuloma, Plasma Cell/complications/*pathology ; Humans ; Intercostal Nerves/*pathology ; Lung Diseases/etiology/pathology ; Middle Aged ; Paraneoplastic Syndromes/etiology/*pathology ; Pemphigus/etiology/*pathology ; Peripheral Nervous System Neoplasms/complications/*pathology

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

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

PURPOSE: Schwannoma, a benign peripheral nerve tumor, is slow-growing, encapsulated neoplasm that originates from the Schwann cell of the nerve sheath. Schwannoma most frequently involves the major nerve. Schwannoma occurring in the superficial radial nerve rare. This is a report of our experience with schwannoma arising from the superficial radial nerve with neurologic symptom. METHODS: A 55-year-old woman presented with eight-month history of progressive numbness and paresthesia in dorsum of the thumb and index finger. Physical examination revealed a localized mass on the midforearm. Sonographic examination showed an ovoid, heterogenous, hypoechoic lesion, located eccentrically in related to the superficial radial nerve. The lesion was mobile in the transverse but not in the longitudinal axis of the nerve, which was thought to favour schwannoma rather than neurofibroma. At operation, a 20 x 15 mm ovoid, yellowish grey mass was seen arising from the superficial radial nerve. The tumor present as eccentric masses over which the nerve fibers are splayed. Using operating microscope, the tumor was removed, preserving the surrounding nerve. RESULTS: Histology confirmed that the mass was a benign schwannoma. There were no postoperative complications. After two months the patient had no clinically demonstrable sensory deficit. CONCLUSION: An unsusual case of a schwannoma of the superficial radial nerve is presented. In case with neurologic symptom, prompt surgical decompression must be made to prevent further nerve damage and to restore nerve function early.
Axis, Cervical Vertebra ; Decompression, Surgical ; Female ; Fingers ; Humans ; Hypesthesia ; Middle Aged ; Nerve Fibers ; Neurilemmoma ; Neurofibroma ; Neurologic Manifestations ; Paresthesia ; Peripheral Nervous System Neoplasms ; Physical Examination ; Postoperative Complications ; Radial Nerve ; Thumb

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

PURPOSE: In treating schwannoma patients, it is critical to determine the origin of the tumor to preserve nerve function. We evaluated the validity of preoperative imaging studies in distinguishing the neurological origin of the schwannomas of the head and neck, and the efficacy of intracapsular enucleation in preserving nerve function. MATERIALS AND METHODS: In 7 cases of schwannomas in the head and neck region, we predicted whether the tumor originated from the vagus nerve or the cervical sympathetic chain through imaging studies including computed tomography (CT) and magnetic resonance imaging (MRI). All patients were performed intracapsular enucleation, and the function of the vagus nerve and the sympathetic nerve was evaluated preoperatively and postoperatively. RESULTS: Preoperative imaging studies showed 6 cases where the tumor was located between the carotid artery and the internal jugular vein, and 1 case where the tumor was located posteriorly, displacing the carotid artery and the internal jugular vein anteriorly. At the time of operation, we confirmed schwannoma originating from the vagus nerve on the first 6 cases, and schwannoma originating from the sympathetic nervous system on the last case. All patients went through successful intracapsular enucleation, and of the seven schwannoma cases, 6 patients maintained normal postoperative neurological function (85.7%). CONCLUSION: Preoperative imaging studies offer valuable information regarding the location and origination of the tumor, and intracapsular enucleation helped us to preserve the nerve function.
Aged ; Diagnostic Imaging/methods ; Female ; Follow-Up Studies ; Head and Neck Neoplasms/complications/diagnosis/*pathology ; Humans ; Magnetic Resonance Imaging/methods ; Male ; Middle Aged ; Neurilemmoma/complications/diagnosis/*pathology ; Peripheral Nervous System/injuries/physiology ; Sympathetic Nervous System/physiology ; Tomography, X-Ray Computed/methods ; Treatment Outcome ; Vagus Nerve/physiology