Granular cell tumour of the ulnar nerve in a young adult

Granular cell tumours in peripheral nerve trunks are extremely rare. We report a case of a granular cell tumour of the ulnar nerve just distal to the elbow in a 16-year-old man. The appearance of the intraneural tumour could not be differentiated by magnetic resonance imaging (MRI) from more common nerve tumours. At exploration it was impossible to excise the tumour radically. Microscopic examination showed a clearly benign tumour and no malignant transformation has been found at follow-up after three and a half years.     

Granular cell tumour of the ulnar nerve

Although granular cell tumours have been demonstrated to have a neural origin, they rarely arise in peripheral nerve trunks. We report a case of granular cell tumour of the ulnar nerve in a 51-year-old man. Though dissectable from the nerve, this intraneural tumour showed microscopic involvement of focal nerve fibres. This tumour tended to infiltrate the nerve in the same manner as a neurofibroma.     

Retroperitoneal Peripheral Nerve Sheath Tumour of Triton Type—A Case Report

The malignant peripheral nerve sheath tumour is the malignant counterpart to benign soft tissue tumours such as neurofibromas and schwannomas. Malignant Triton Tumour is a histological variant of malignant peripheral nerve sheath tumours with rhabdomyosarcomatous differentiation. They are known to develop in cases of neurofibromatosis type 1 (von Recklinghausen’s disease). Malignant peripheral nerve sheath tumor is biologically an aggressive tumour for which the treatment of choice is surgery. Here we report a rare case of malignant peripheral nerve sheath tumour, not associated with neurofibromatosis type 1 and arising in the retroperitoneum.     

Peripheral nerve tumour composed of neurilemmoma and haemangioma elements

Summary Five cases of a peripheral nerve tumour with both neurilemmoma and haemangioma components are reported.Three tumours were intraspinal, one tumour originated in the acoustic nerve, and one was in the brachial plexus.Because of rapid progression these mixed tumours should be considered in the differential diagnosis of intraspinal malignancies.     

Carotid body tumours: the University of Alberta Hospital experience

At the University of Alberta Hospital between 1950 and 1988, 17 patients who had a diagnosis of carotid body tumour were seen; 15 of them were followed up for an average of 8 years (range from 1 to 38 years). In 14 patients the tumour was removed surgically. There were no operative deaths and no strokes occurred. The most frequent complication was cranial nerve deficit. Of the 15 patients followed up, 10 (67%) manifested a deficit of the facial, vagus or hypoglossal nerve. The primary tumour was diagnosed histologically as a benign neoplasm in all 14 patients operated on, but in 3 distant metastases developed or there was invasive local recurrence. Patients with malignant tumour were significantly (p less than or equal to 0.01) younger than those with a benign tumour. Carotid body tumours can be managed safely with respect to stroke complications, but cranial nerve injuries continue to be a problem. Malignant tumours are difficult to distinguish from benign tumours except that they tend to occur in younger patients. Prompt surgery and close follow-up is particularly important in patients with carotid body tumour.     

Oculomotor Nerve Neurinoma: Report of two Cases

Summary  Being motor nerves neurinomas originating from ocular nerves are very rare, unless associated with neurofibromatosis. Authors describe two cases of oculomotor nerve i.e. third nerve, Schwann cell tumours. One of them presented as a cavernous sinus mass in a middle aged lady while the other was a middle aged man with a large cisterno-cavernous tumour. Surgical approach is discussed and the relevant literature reviewed.     

Personal treatment of a case of gastrointestinal autonomic nerve tumour of the second duodenal segment

Gastrointestinal autonomic nerve tumours are an uncommon form of gastrointestinal stromal tumours. Since both gastrointestinal stromal tumours and gastrointestinal autonomic nerve tumours are potentially malignant, radical surgical excision is always required. We report a case of a gastrointestinal nerve tumour measuring about 5 cm in diameter and arising from the medial wall of the second portion of the duodenum about 1.5 cm below the papilla of Vater. Because of this rare location a very invasive procedure (duodenocephalo-pancreatectomy) might have been required for tumour resection. We avoided this operation and implemented an alternative solution. Endoscopic ultrasonography was very helpful for this purpose, revealing that the tumour was confined within the duodenal wall and separated from the papilla. We employed a non-conventional surgical technique consisting in a duodenal resection comprising the tumour and a direct TT anastomosis of the duodenal stumps. Two technical devices were of fundamental importance for carrying out this procedure: (i) Ligasure which made dissection between the pancreatic head and duodenal wall a safe manoeuvre with little bleeding; and (ii) Valtrac, which allowed us to perform a large anastomosis without any tension on the duodenal stumps. Intraoperative endoscopy was also important. No anastomotic leakage occurred. At follow-up at 12 months the patient is in good health and CT scan and endoscopic ultrasonography have shown no recurrence of disease.     

Prediction of Facial Nerve Displacement in Extralarge Vestibular Schwannoma

Summary  The primary objective in the surgery of extra large vestibular schwannoma is the total removal of the tumour mass while preserving the facial nerve. Preservation of the facial nerve in extra large tumours is reported as being notoriously difficult in the majority of cases  This study was undertaken to evaluate the accuracy in predicting displacement of the facial nerve by preoperative radiological imaging studies in 19 cases of extra large vestibular schwannoma. The direction of displacement of the facial nerve was predicted with preoperative axial and coronal MRI scans and verified intra-operatively.  We achieved total removal of tumours in 84.2%, facial nerve displacement was predicted in 80% and we accomplished anatomical preservation in 80%. Prediction of displacement was difficult in tumours with little or no intracanalicular components or with severe bony destruction of the internal acoustic meatus.  The preoperative prediction of facial nerve displacement in extra large tumours allows safe internal decompression of the tumour and careful dissection near the predicted area of the facial nerve during the operation. Consequently, a high rate of anatomical preservation of the facial nerve can be achieved.     

Giant cell tumour of the sphenoid bone with coincidental galactorrhoea--a case report

A case of the rare giant cell tumour involving the sphenoid bone is reported. The usual presentation of these tumours is headache and cranial nerve deficits. This 25-year-old lady presented with oligomenorrhoea and galactorrhoea. Investigations demonstrated an asymptomatic erosive mass lesion in the sphenoid with suprasellar extension and extension into the nasopharynx. The tumour was partially resected via the transphenoidal route. The differential diagnosis and treatment of these lesions are discussed.     

Criteria for preservation of vestibulocochlear nerve function during microsurgical removal of acoustic neurinomas

Summary A careful examination of the shape, location and course of the 8th cranial nerve in medium-sized and large tumours exhibits three distinct variants. In all cases where cochlear function was preserved, the type III variant (12%) in the cranial nerve tumour relationship was observed. The author's experience clearly indicates that, if a tumour is resected in toto, anatomical nerve continuity cannot be preserved in the type I and II variants (48%, respect. 40%). However, in the type I and II variants the patients invariably had preoperative hearing loss. The important criteria which must be considered in order to preserve cochlear function when extirpating acoustic neurinomas are an anatomically intact nerve, the origin of the tumour and its direction of spread, further more the shape, location and course of the 8th nerve components, the quality of preoperative cochlear nerve function, the patttern of vascularization of the statoacoustic nerve and the inner ear, and lastly, but not least a possible infiltration of the vestibular and/or cochlear nerves by the tumour itself. Objective hearing function could be preserved in 62% of small neurinomas (grade II) and 10% of large tumours (grades III and IV).     

Testicular and paratesticular tumours in children: 30 years' experience.

BACKGROUND: Testicular or paratesticular tumours in children are rare, making it difficult to achieve the best management for these life-threatening diseases. The aim of this study is to review patients during a 30-year period with these tumours and assess clinical aspects to improve management. METHODS: The records of 68 patients from 1967 to 1996 were reviewed with respect to age at diagnosis, affected sites, presentation, clinical diagnosis, operation, pathology and prognosis. RESULTS: The most common presentation was a painless scrotal mass (84%). The most common testicular tumour was mature teratoma (n = 27) followed by yolk sac tumour (n = 17). Thirteen patients had paratesticular rhabdomyosarcoma. Two teratocarcinomas, three leydig cell tumours, two sertoli cell tumours, one granulosa cell tumour, one fibroma, one gonadoblastoma, and one secondary tumour from acute myeloid leukaemia were found also. Testis-sparing surgery was performed in 21 of 33 patients with benign tumours (27 teratoma, three leydig cell tumours, two sertoli cell tumours, one fibroma), which caused no recurrence. Only two patients with rhabdomyosarcoma and one with mixed germ cell tumour died of their disease. CONCLUSION: Recent combined therapy with surgery and chemotherapy against primary testicular and paratesticular tumours has improved prognosis. Testis-sparing surgery should be considered for benign tumours.     

Giant vestibular schwannomas: focusing on the differences between the solid and the cystic variants

In vestibular schwannomas (VS), the tumour size, as well as the size of the cystic component, have a considerable bearing on the outcome. This study addresses the differences between the cystic and solid variants of giant vestibular schwannomas. The study included 62 patients with giant VSs, of which 40 were solid and 22 were cystic (those in which cystic component greater or equal to 30% of the total tumour volume). The cystic tumour group was further divided into type A (31-60% volume of the cyst within tumour), type B (61-90% intra-tumoural cyst volume) and type C (more than 90% volume of the cyst). The clinicoradiological features, operative findings, histopathological characteristics and outcome of surgery of the two groups were compared. The mean duration of symptoms for the solid and cystic tumours were 21.1 and 26.2 months, respectively. However, six patients with cystic tumours showed recent and rapid neurological deterioration after a protracted existence. Papilloedema, lower cranial nerve involvement, facial paraesthesias and preoperative hydrocephalus were significantly more in cystic tumours. Total excision was achieved in 38 of the solid and 18 of the cystic tumours. VIIth nerve preservation was higher in the cystic lesions [solid 33/40 (82.5%), cystic 21/22 (95.4%)]. Myxoid degeneration, lobular growth patterns and cellular atypia were more prominent in the cystic variants. The giant vestibular schwannomas were associated with a higher incidence of cystic degeneration than has been reported for smaller tumours in literature. In cystic lesions, VIIth nerve preservation was higher due to early decompression of the lesion that facilitated in early identification of the VIIth nerve, except in patients with type C cystic tumour.     

One very rare and one new tracheal tumour found by electron microscopy: glomus tumour and acinic cell tumour resembling carcinoid tumours by light microscopy.

Tracheal tumours were removed surgically from two patients and diagnosed as carcinoid tumours by routine light microscopy. At a later date, electron microscopy was performed on stored tumour tissue and no neurosecretory granules were found in either case. One showed features of a glomus tumour and the other of an acinic cell tumour. Only two glomus tumours appear to have been reported previously in the trachea, and no acinic cell tumours. Electron microscopy is thus sometimes of great assistance in diagnosing accurately unusual tumours of the lower respiratory tract.     

Regrowth of the residual tumour after acoustic neurinoma surgery

Ideally, acoustic neurinomas should be completely removed without neurological deficit. In some cases, however, removal is subtotal in order to preserve anatomical continuity of the facial nerve. In this report we discuss the regrowth of residual tumour in these patients and the difference between regrowing and dormant tumours. Eight of 81 patients with acoustic neurinomas where the facial nerve was anatomically preserved leaving tumour overlying it were investigated and followed-up. Only one case with residual tumour along the 7th nerve from the intrameatal portion to its entry to the brainstem showed regrowth 3.5 years after the operation. In the other seven cases with residual tumour along the nerve from the porus to the brainstem, the tumour remained dormant during a follow-up period of 4.5-8.5 years. Removal of tumour in the internal auditory meatus may make regrowth unlikely.     

De novo malignant peripheral nerve sheath tumor of the breast: case report number one

Summary BACKGROUND: Sarcomas of the breast are rare, representing less than 1% of malignant breast tumours. Malignant peripheral nerve sheath tumour (MPNSTs) is the malignant counterpart to benign soft tissue tumours such as neurofibromas and schwannomas. It is the most common sarcoma arising in the setting of von Recklinghausen's disease. METHODS: We report a de novo malignant peripheral nerve sheath tumour of the breast in 18-year-old patient. To the best of our knowledge, this is the first reported case of sporadic MPNST occurring in the breast in the absence of neurofibromatosis. RESULTS: Immunohistochemical examination is essential to establish the diagnosis and helpful in excluding other lesions in the differential diagnosis. CONCLUSIONS: The surgeon should approach these tumours according to the established guidelines for soft tissue sarcoma surgery.      

Gastric stromal tumors. Results of a multicenter study. French Associations of Surgery Research

AIM OF THE STUDY: Gastric stromal tumours are not perfectly known. The aim of this retrospective multicenter study (29 centers) was to improve knowledge of these tumours. PATIENTS AND METHODS: From 1986 to 1994, 159 patients were operated on for leiomyomas (50), leiomyosarcomas (24), malignant/benign schwann cell tumours (10/29), automatic nerve tumours (4), leiomyoblastomas (28), spindle cell tumours (14). The mean duration of follow-up was 5 years. Presenting symptoms, diagnostic procedures, operative and pathological findings, evolution (recurrence, death) were recorded for each patient. RESULTS: Gastrointestinal bleeding and epigastric pain were the most common presenting symptoms (54% and 50% of patients, respectively). Endosonography was the most sensitive examination (97%). Malignant tumours size was greater than benign tumours size (12.6 cm versus 5.2 cm). Extension to contiguous organs or metastases were frequent (33% and 26% of patients, respectively). In 16 patients, pathological examination could not differentiate between malignant and benign tumour. Seven patients who had been operated on for a benign tumour (6%) developed a local (n = 4) or a metastatic (n = 3) recurrence. The 5-year survival rate was 40% for leiomyosarcomas, 28% for schwannosarcomas and 90% for malignant leiomyoblastomas. CONCLUSION: The main feature of stromal gastric tumours is the frequent difficulty to differentiate between malignant and benign tumours. The prognosis of malignant tumours depends on pathological types. The prognosis of benign tumours is uncertain since recurrences may develop.     

Isolated peripheral neurilemoma attached to the tendon of the flexor digitorum longus muscle

Neurilemomas are benign tumours arising from peripheral nerves with a Schwann cell sheath. They are normally painless and slowly growing, rarely causing motor disturbances. Neurilemomas are most common in the cranial nerves, in the trunk, the upper and lower extremities, but may appear anywhere. Especially rare are neurilemomas of the lateral peroneal nerve in the region of the fibular head and in the foot. We present the first detailed report of a neurilemoma localized between the Achilles tendon and the flexor digitorum longus muscle with separation of the tumour from the tibial nerve.     

Neuroendocrine characteristics of human Leydig cell tumours

Summary. The neuroendocrine nature of a subset of Leydig cells has already been established. The present investigation deals with neuroendocrine characteristics of Leydig tumour cells. A number of neuroendocrine and neuronal markers were demonstrated in Leydig cell tumours of 7 men aged 25–41 years. The following substances were immunocytochemically tested in Leydig tumour cells: the monoamine-synthesizing enzymes tyrosine hydroxylase and aromatic L-amino acid decarboxylase, the indoleamine serotonin, the calcium-binding protein parvalbumin, the microtubule associated protein-2, neurofilament protein 200, synaptophysin, neuron specific enolase, substance P and neuronal nitric oxide synthase (NOS). Compared to the normal interstitial cells beyond the tumours, all neoplastic cells showed a significantly weaker immunoreactivity for nerve cell markers as well as for testosterone and cyclic guanosine monophosphate (cGMP), which is usually accumulated by nitric oxide (NO). This provides evidence for a certain dedifferentiation of Leydig tumour cells.
However, these results suggest that tumourous development of Leydig cells does not include loss of neuronal phenotype. Moreover, on the assumption that 'neuronal' Leydig cells exist beside 'non-neuronal' ones in normal testicular tissue, we propose the hypothesis that 'neuronal' Leydig cells can transform to tumour cells.     

TESTICULAR AND PARATESTICULAR TUMOURS IN CHILDREN: 30 YEARS’ EXPERIENCE

Background : Testicular or paratesticular tumours in children are rare, making it difficult to achieve the best management for these life-threatening diseases. The aim of this study is to review patients during a 30-year period with these tumours and assess clinical aspects to improve management. Methods : The records of 68 patients from 1967 to 1996 were reviewed with respect to age at diagnosis, affected sites, presentation, clinical diagnosis, operation, pathology and prognosis. Results : The most common presentation was a painless scrotal mass (84%). The most common testicular tumour was mature teratoma (n = 27) followed by yolk sac tumour (n = 17). Thirteen patients had paratesticular rhabdomyosarcoma. Two teratocarcinomas, three leydig cell tumours, two sertoli cell tumours, one granulosa cell tumour, one fibroma, one gonadoblastoma, and one secondary tumour from acute myeloid leukaemia were found also. Testis-sparing surgery was performed in 21 of 33 patients with benign tumours (27 teratoma, three leydig cell tumours, two sertoli cell tumours, one fibroma), which caused no recurrence. Only two patients with rhabdomyosarcoma and one with mixed germ cell tumour died of their disease. Conclusion : Recent combined therapy with surgery and chemotherapy against primary testicular and paratesticular tumours has improved prognosis. Testis-sparing surgery should be considered for benign tumours.     

Malignant epithelial parotid tumours

Formal parotidectomy was performed in 120 patients by one surgeon over 7 years. Eighteen patients were referred with malignant salivary tumours (eight recurrent). Low-grade tumours (LGT) were treated by surgery alone; untreated high-grade tumours (HGT) were treated by pre- or post-operative radiotherapy according to clinical findings. The aim of surgery was to obtain tumour clearance, to preserve the facial nerve where possible, and to perform radical neck dissection for HGT when peroperative jugulodigastric lymph node biopsy confirmed metastasis. Five patients (all HGT) with complete facial palsy due to tumour underwent radical parotidectomy; of the remainder, only one suffered further deterioration of facial nerve function after surgery. After 5 years median follow-up from presentation, four patients with HGT have died from metastases; one has developed local recurrence. No patient with LGT has died or developed local recurrence. The survival difference between patients with HGT and LGT is statistically significant (P less than 0.05). A logical management policy for malignant parotid tumours requires knowledge of tumour grade.