Carpal tunnel syndrome due to fibrolipomatous hamartoma of the median nerve

Nervous lipofibromatous hamartoma is a rare tumor-like condition involving the peripheral nerves, whereby the epineurium and perineurium are enlarged and distorted by excess of fatty and fibrous tissues that infiltrate between and around nerve boundaries. The median nerve is much more likely to develop a hamartoma than other nerves with a predilection for the carpal tunnel. We present a case of carpal tunnel syndrome in an adult caused by fibrolipomatous hamartoma of the median nerve, successfully removed by excision of the fibrolipomatous tissue and decompression.     

Fibrolipoma of the median nerve

Neural fibrolipoma or fibrolipomatous hamartoma is an uncommon benign tumor that usually arises in the median nerve. Fibrofatty tissue proliferates around the nerve and infiltrates the epineurium and perineurium. We report a case of fibrolipomatous hamartoma of the left median nerve in an 18-year-old woman. Our objective was to describe the pathognomonic magnetic resonance imaging features, whose presence obviates the need for a diagnostic biopsy.     

Nerve tumors of the hand and forearm.

Peripheral nerve tumors comprise less than 5% of all tumors of the hand. The most common solitary nerve tumor is the neurilemmoma, which arises from the neural sheath, is well encapsulated, minimally symptomatic, and may be surgically enucleated without producing a neurological deficit. Neurofibromas may be solitary, multiple, or associated with von Recklinghausen's disease. They are usually centrally placed with nerve fibers traversing the tumor mass making it more difficult to remove the tumor without producing permanent neurological damage. Malignant tumors include neurofibrosarcomas which often are very aggressive, requiring wide excision or amputation, and the rare neuroepitheliomas. Reported nerve tumors, intraneural in location but nonneural in origin, include fibrofatty infiltration of the median and digital nerves, intraneural lipoma, hemangioma, and ganglion cysts. These lesions may be treated by decompression or excision, depending on the nature of the tumor. Four unusual cases are described.     

Recurrent carpal tunnel syndrome in a child due to fibrolipomatous hamartoma of the median nerve successfully treated by limited excision and decompression.

We present a case of recurrent carpal tunnel syndrome in a child caused by fibrolipomatous hamartoma of the median nerve which was successfully treated by limited excision of the fibrolipomatous tissue and decompression.     

Carpal tunnel syndrome caused by a fibrolipomatous hamartoma of the median nerve treated by endoscopic release of the carpal tunnel

Fibrolipomatous hamartoma has up to now been considered a rare anomaly that most commonly affects the median nerve. Its pathogenesis is controversial. The magnetic resonance (MR) appearance is pathognomonic and precludes the necessity for a diagnostic biopsy. Its reported incidence is likely to increase as a result of the increased use of MRI. There is no definitive treatment, although carpal tunnel syndrome caused by fibrolipomatous hamartoma has been noted to respond to open release of the carpal tunnel. We describe a case of carpal tunnel syndrome caused by fibrolipomatous hamartoma of the median nerve that responded to a two-port endoscopic technique of release. Provided that a safe, distinct surgical plane can be established between the median nerve and the flexor retinaculum, the two-port technique of endoscopic release of the carpal tunnel is both safe and effective.     

Intraneural Lipoma of the Tibial Nerve: A Case Report

Intraneural lipomas, neurofibrolipomas, lipofibromatous hamartomas, and perineural lipomas are subsets of hamartomas that typically present as fibroadipose, soft tissue masses within the epineurium of a nerve. Several cases involving intraneural lipomas of the median nerve in the upper extremity have been reported; however, owing to the lesion's rare incidence in the foot and ankle, only a select few cases involving the superficial peroneal nerve have been reported. We present the first case of a tibial nerve intraneural lipoma in a 42-year-old female with a follow-up period of 2 years. We discuss the clinical presentation, distinguishing features, surgical procedures, and short-term outcome regarding this unique tumor.     

Fibrolipomatous hamartoma of the median nerve

A case of fibrolipomatous hamartoma of the median nerve is presented. The operative findings were characteristic. The diagnosis should be confirmed by histological examination of a biopsy from an involved palmar cutaneous branch, and treatment should be limited to decompression of the ligament or fascia over the involved area.     

Intraneural lipoma of the sciatic nerve

A large, well-encapsulated intraneural lipoma occurred within the sciatic nerve of a 34-year-old woman. In a review of the literature, significant differences were noted between the well-encapsulated and the diffusely infiltrative lipofibromatous hamartoma types of intraneural lipomas. The average age at the time of appearance of the well-encapsulated type is 45 years, with female predominance, while the infiltrative type arises in a younger age group (average age, eight years) with no sexual predominance. To avoid a recurrence, total excision is recommended in cases of the well-encapsulated type. Attempts to excise the diffusely infiltrative type are likely to fail.     

Intraneural ganglion cyst of the ulnar nerve in an unusual location: A case report

IntroductionIntraneural ganglion cysts are benign, mucinous, non-neoplastic lesions of the peripheral nerves. While the most common location of intraneural ganglion cysts is the ulnar nerve and its branches, intraneural ganglion cyst involving the superficial branch of the ulnar nerve has not yet been reported.Presentation of caseA-25-year-old woman presented with pain and a palpable mass in the hypothenar region of the volar side of her right hand. Her neuromuscular examination was normal. The pain was unresponsive to nonsurgical treatments. After confirming with imaging modalities, the initial diagnosis was considered as an intraneural ganglion cyst arising from superficial ulnar nerve. Excision of the ganglion and exploration of the articular branch (if seen in operation) decision was undertaken by the senior author. Whether MRI or intraoperative exploration, not identified an articular branch.DiscussionIntraneural ganglion cysts of peripheral nerves may be seen in miscellaneous locations in the body. However, to our knowledge, an intraneural ganglion cyst involving the superficial branch of the ulnar nerve is unique. While a variety of theories have been proposed to enlighten the etiopathogenesis of intraneural ganglia, the latest and most affirmed is the unifying articular (synovial) theory.ConclusionIntraneural ganglion cysts may be seen on the hypothenar side of the palm. The etiology and treatment of choice are closely associated with each other in this rare disorder. It is important to realize a related articular branch, otherwise the origin of cyst formation remains, and this may cause other para-articular cysts.     

Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

A three-year-old boy was investigated for inexplicable incessant crying. On examination, his left wrist was mildly swollen (three to four months) and sensitive. Exploration and carpal tunnel decompression of the left wrist with incisional biopsy was performed for the presence of a fusiform swelling intimately associated with the median nerve. Histopathology revealed the presence of enlarged nerve bundles admixed with mature fat cells and diffuse fibroblastic proliferation. Three months later, he underwent urgent contralateral carpal tunnel decompression for a similar presentation. The final diagnosis was bilateral fibrolipomatous hamartoma (FLH) of the median nerves causing acute bilateral compression neuropathy.FLH of the median nerve is an extremely unusual cause of acute bilateral carpal tunnel syndrome in a young child presenting with ‘incessant crying’. A comprehensive review of FLH including epidemiology, etiology, clinical presentation, differential diagnosis, imaging, pathology, treatment and prognosis is discussed.     

Neurothekeoma of the median nerve: case report

We report the case of a large intraneural neurothekeoma of the median nerve at the wrist. Neurothekeomas are rare; they are small, superficial, and typically asymptomatic benign tumors of undetermined cellular origin. Complete excision is usually curative. This case is interesting owing to the tumor's large size and location within the median nerve, which made it highly symptomatic, mimicking carpal tunnel syndrome.     

骨内脂肪瘤19例回顾分析

目的 :探讨骨内脂肪瘤的诊断和治疗策略。方法 :回顾性分析自1986年6月至2016年1月收治的骨脂肪瘤患者19例,男12例,女7例;年龄24~76岁,其中13例年龄为40~50岁。因疼痛、肿胀就诊15例,体检偶然发现3例,另1例为骨囊肿刮除术后4年发生骨缺损脂肪瘤替代。X线片表现为边界清楚的溶骨性破坏,CT或MRI显示脂肪当量的病损,能术前确诊。其中16例采用手术治疗,3例采用保守治疗。结果:19例患者均获得随访,时间9~42个月,平均15个月。手术治疗的16例患者无临床或放射学信号复发,3例保守观察的患者病变无扩大。结论:对确诊而无临床症状的骨内脂肪瘤应采取保守观察,有明确症状的患者应积极手术病灶刮除植骨。     

Free dermal fat graft for coverage of the median nerve following failed carpal tunnel release

ObjectivesWe wished to evaluate the effectiveness of a free dermal fat graft for covering the median nerve in order to improve function after failure of carpal tunnel release.MethodsEight patients with nine failures of carpal tunnel release were reviewed for this retrospective study. They all had significant perineural fibrosis. We performed a free dermal fat graft harvested from the inguinal region on the same side. The mean follow-up was 37 months (range 6 to 112). All patients had a clinical examination, a disability arm and shoulder scoring (DASH) and magnetic resonance imaging (MRI).ResultsFive patients were satisfied or very satisfied with the procedure although only two patients returned to work. Mean grip strength was 13 kg (43% of non-operated hand) and the two-point discrimination test was normal or subnormal six times on the eight wrists tested. The mean DASH score was 43 out of 100. MRI scans on eight wrists showed a fat signal in all patients and enhanced signal with gadolinium contrast in six cases.ConclusionsFree dermal fat graft offers a good covering for the median nerve over the carpal tunnel in cases of failure of carpal tunnel release. MRI showed viability of the graft. Clinical results show improvement but symptoms of median nerve irritation persisted. Permanent intraneural changes may explain these limited results.     

Paragangliomes de la queue de cheval : à propos de six cas et revue de la littérature

Background and purposeParagangliomas of the cauda equina are rare tumors. The standard treatment is surgical resection. Our study aims to compare our clinical, radiological, prognostic data to the literature and to offer management and follow-up recommendations.MethodsIn this retrospective study, six patients with paraganglioma of the cauda equina region were treated. Symptoms included radicular nerve pain and low back pain with occasional sphincter dysfunction and motor deficit. MRI showed well-circumscribed lesions with homogeneous enhancement following gadolinium injection. Treatment involved complete surgical resection of the tumor under electrophysiological control. In addition to the characteristics of the tumor, we assessed operating results as well as postoperative morbidity and follow-up.ResultsAll patients had complete removal of the tumor, which required in most cases the resection of the carrying root. The intervention allowed a regression of the initial symptoms, with possible postoperative regressive sphincter disorders. Clinical and radiological follow-up (19 months on average), showed no tumor recurrence.ConclusionsThe reference treatment of these tumors is complete surgical resection, usually requiring the sacrifice of the carrying nerve root. Intra-operative nerve roots stimulation is recommended to reduce the risk of motor deficit linked to this radical treatment. A long-term clinical and radiological follow-up is recommended.     

Characterization of two distinct lipomas: a comparative analysis from surgical perspective

Background: Lipomas are common benign soft tissue tumors that are well-circumscribed and encapsulated. However, adipose masses that are not demarcated from the surrounding fat are often encountered. Two distinct types of lipomas were analyzed from surgical perspective.

Methods: Thirty patients were enrolled after lipoma excision and diagnosed with either encapsulated (n?=?20) or non-encapsulated lipoma (n?=?10). Comparison of clinical variables, histologic analyses and characterization of the lipoma adipose-derived stem cells (ASCs) between the two lipomas were performed.

Results: Non-encapsulated lipomas were associated with older age at operation, larger tumor and increased seroma formation. The density of lymphatic vessels and gene expressions related to lymphatic vessel, inflammation and proliferation were increased in non-encapsulated lipoma. ASCs of non-encapsulated lipoma showed enhanced proliferation when cultured with serum.

Conclusions: Non-encapsulated lipomas and their ASCs showed distinct lymphatic histology and cellular response. These findings elucidated the pathogenesis and pathophysiology of lipomas.     

Carpal tunnel syndrome caused by a giant lipoma of the hand: A case report

Introduction and importanceLipomas are common benign tumours which occur in up to 2% of the population. They are classified as giant when larger than 5 cm. Although they are usually asymptomatic, giant lipomas of the hand may cause compression of the underlying tissues.Case presentationA 62-year-old Caucasian male presented to the Plastic and Reconstructive Surgery outpatient clinic with numbness and pain in his left hand. The numbness in his fingers pointed to compression of the median nerve, as well as the ulnar nerve. He presented with a rapidly progressive swelling in his left palm. An MRI scan of the hand was made, which showed a lipoma of approximately 8,5 cm in diameter. The swelling was surgically removed and sent for histopathological analysis, which confirmed the diagnosis of benign giant lipoma of the hand. Two weeks postoperatively, pain and numbness significantly decreased.Clinical discussionNeural injury in carpal tunnel syndrome is related to the duration and degree of compression. A giant lipoma is considered malignant until proven otherwise since variants with high potential for metastasizing exist. Distinguishing between a benign tumour and a malignant lipoma is essential, since a more radical treatment plan might be required.ConclusionGiant lipomas of the hand are a rare cause of carpal tunnel syndrome and a malignant variant should always be suspected. A preoperative MRI scan should be performed. Rapid en bloc excision is necessary in case of compression of the underlying tissues.     

Multicystic adenomatoid pancreatic hamartoma in a child: Case report and literature review

INTRODUCTIONPancreatic tumor is a rare condition in children, but reasonably common in adults. Histopathology in children also differs from that in adults, with most cases being pancreatoblastoma, solid pseudopapillary tumor, or pancreatic endocrine tumors.PRESENTATION OF CASEA 14-month-old boy was noticed abdominal distension and referred to our hospital. Laboratory findings revealed leukocytosis and elevation of serum level of C-reactive protein and pancreatic enzymes. Radiological findings at admission were the huge abdominal cyst in abdominal computed tomography. As the levels of pancreatic enzyme elevated synchronous to oral feeding, total parenteral nutrition was needed. Besides radiographically the abdominal lesion changed from multiple large cystic type to multiple microcystic lesion including solid component over time. It was considered different diagnosis was pancreatic blastoma, pancreatic pseudo cyst, and lymphangioma and he was performed operation. The huge multicystic and partially solid tumor arising from the tail of pancreas existed from posterior of stomach to pelvic cavity. The tumor was completely resected without pancreatectomy and residual pancreas can be kept without tumor invasion. Histopathological finding was pancreatic hamartoma.DISCUSSIONPancreatic hamartoma was extremely rare, and only 17 cases were previously reported in the literature. This is the first case that the change of radiographic findings overtime was shown. We reviewed 17 cases (4 cases in children) of pancreatic hamartoma including our case.CONCLUSIONAlthough extremely rare, pancreatic hamartoma should be considered in the differential diagnosis of cystic abdominal mass in children.     

A case report of hibernoma in the thigh comprising the femoral neuromuscular bundle

IntroductionHibernomas are rare, slow-growing, painless, benign tumors of soft tissue that develop from residual brown fat cell showing predilection for areas where brown fat is more common in fetuses and infants. Due to the rarity of the tumor its often overlooked or mistaken for other pathologies such as liposarcoma.Case presentationUsing the CARE 2020 criteria we describe a 26-year-old man who presented with painful swelling of the thigh. A magnetic resonance imaging examination without contrast showed a mass in the medial thigh just lateral to the femoral artery. A biopsy specimen was obtained from his thigh for histopathological analysis, and the findings suggested a hibernoma. A wide resection was performed, and it showed that the femoral nerve was lateral to the mass, with the course of the nerve altered due to mass effect. The mass was sent for a histopathological examination, and the findings were consistent with a hibernoma. Two weeks post-operative, the patient reported a considerable decrease in pain intensity, and six months post-operative, he reported complete pain relief.DiscussionHibernomas are unusual tumors that are benign and usually painless, but the large size the tumor can reach and proximity to vital structures can explain the growing trends symptomatic nature of some hibernomas. A proper and step wise approach using clinical, radiological and histopathology is important to Diagnose and plan surgical management. Proper understanding of the proximity of adjacent structures and the high vascularity of the tumor.ConclusionBecause of the rarity and under reporting of hibernomas they are often misdiagnosed as large lipomas or malignant soft tissue tumors, underscoring the importance of histopathology in ensuring accurate diagnosis and anticipating intraoperative. Careful dissection and ligation of the vasculature with the understanding of adjacent anatomy are key to safe tumor excision.     

Paraspinal plexiform schwannoma of unknown nerve origin: A case report

Introduction and importanceSchwannomas are benign, slow-growing nerve sheath tumors of neoplastic Schwann cells. They are the most common peripheral nerve tumors in adults and are typically discovered incidentally due to their asymptomatic presentation. Despite the fact that most schwannomas are unassociated with a syndrome, their etiology is thought to be related to alterations or loss of the neurofibromatosis type two tumor suppressor gene.Case presentationWe present the case of a fifteen-year-old female who presented with a recurrent lower back/upper buttocks 9 cm mass with imaging suspicious for schwannoma. Needle biopsy revealed an S100 positive cellular schwannoma with patchy Ki-67. During surgical dissection down to the sacrum, no nerve of origin was identified.Clinical discussionSchwannomas have no pathognomonic findings on MRI and may occur at any location that Schwann cells are present; therefore, confirming a diagnosis relies on histopathology. Plexiform schwannomas are defined by a “network-like” intraneural growth pattern and are exceedingly rare in paediatric populations. A location distinct from the spinal canal is also very rare as schwannomas typically originate from the head and neck region.ConclusionPaediatric plexiform schwannomas have been rarely reported. Surgical planning relies on multiple factors such as tumor size, tumor location, pathologic features and symptomatic burden. The distinctive features of this case including an unknown nerve origin and a location outside the spinal canal provide a unique opportunity to discuss the diagnosis and management of paraspinal schwannomas and the impact on operative planning when a nerve of origin is not identified.     

Fibrolipomatous hamartoma of nerve. A clinicopathologic analysis of 26 cases

Nineteen cases of fibrolipomatous hamartoma of nerve without macrodactyly and seven cases with macrodactyly are discussed. Twenty-five involved the hand, wrist, palm, and finger, and one case involved the foot. Nineteen patients had isolated fibrofatty enlargement of nerve, while seven had macrodactyly in addition to the peripheral nerve changes. Involved nerves included the median nerve, ulnar nerve, an unidentified nerve near the elbow, and a nerve on the dorsum of the foot. Four of nine patients with neurologic symptoms of pain or paresthesias had physical findings compatible with compression neuropathy, and two others were described as having carpal tunnel syndrome. Most patients had been aware of a mass for several years. Microscopically, the lesion was characterized by fibrofatty enlargement of nerve with massive epineural and perineural fibrosis. In two of the cases with macrodactyly, the fibrofatty enlargement of the nerve was associated with overgrowth of bone and the surrounding subcutaneous tissues. In one case, the perineural fibrosis was associated with metaplastic bone. The histogenesis of fibrofatty overgrowth of nerve has been disputed. Mature fat cells have been described within the normal nerve sheath, and it is thought that proliferation of these cells leads to the fatty enlargement of the nerve and its coverings. The relationship of these neural changes to the development of macrodactyly remains controversial. Follow-up in 18 patients (69%) reveals a benign course following biopsy, limited excision, or division of the flexor retinaculum in the wrist.