Adrenal Schwannoma

Background:

Adrenal schwannomas are very rare tumors that are difficult to diagnose preoperatively. We report the case of a left adrenal schwannoma incidentally discovered in a 55-year-old man during a postoperative checkup for a cutaneous malignant melanoma.

Methods:

The biological evaluation was unremarkable, and the radiological examination revealed the adrenal mass that was first considered a metastatic lesion. Adrenalectomy was performed by the laparoscopic approach.

Results:

The postoperative course was uneventful. Histological examination established the correct diagnosis of schwannoma, which was also confirmed by immunohistochemical staining.

Conclusions:

A nonsecreting adrenal mass can be easily misjudged, especially in the context of a recently operated on malignancy. Unilateral adrenal metastasis needs pathological confirmation, as it can dramatically affect prognosis. Unusual tumors of the adrenal gland may be found incidentally, and a malignant context will generate difficulties in establishing the right management. Complete laparoscopic excision is the treatment of choice whenever feasible and will also clarify pathology.     

Management of multiple tumors in neurofibromatosis type 2 patients

Introduction

Neurofibromatosis type 2 is characterized by the presence of bilateral vestibular schwannomas. However, other nervous system tumors may also occur. Therefore, the management of NF2 patients is complex and requires a multidisciplinary discussion in a specialized center.

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.     

Melanocytic Malignant Peripheral Nerve Sheath Tumor of the Male Breast

Summary

Background

Malignant peripheral nerve sheath tumors are rare tumor entities that originate from peripheral nerve sheaths and have an unfavorable prognosis. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. Breast is an absolutely rare location of this lesion, and presentation as a breast lump in the male breast is even rarer.

Case Report

A 65-year-old man presented with a 6-month history of a painless mass of the left breast. Tissue biopsy was performed. Histopathology revealed a malignant spindle cell tumor which was confirmed to be a melanocytic malignant peripheral nerve sheath tumor on the basis of immunopositivity for HMB45 and S-100.

Conclusion

There are no generally accepted guidelines for the treatment of malignant peripheral nerve sheath tumors in the male breast. The patient was referred for radiation therapy after simple mastectomy.Key Words: Malignant peripheral nerve sheath tumor, Breast, Melanocytic     

Dorsal dumb-bell melanotic schwannoma operated on by posterior and anterior approach: case report and a review of the literature

The schwannomas are benign tumors originating from Schwann cells which constitute the nerve sheath. The dorsal pigmented type of schwannomas is relatively rare (Goldstein in Am J Med Genet 86:62–65, 2004; Kurtkaya-Yapicier in Histol Histopathol 18(3):925–934, 2003). There are two distinct types of melanotic schwannoma: the sporadic melanotic schwannomas and the psammomatous melanotic schwannomas of Carney complex. We report a case of a patient harboring a right dorsal dumb-bell melanotic schwannoma and left adrenal mass. The patient underwent a surgical procedure for en bloc total removal of the mass by a posterior and anterior approach. Histopathological examination revealed the diagnosis of melanotic schwannoma. At present, we have no reliable marker of histopathological malignancy of melanotic schwannoma so the follow-up period ought to continue for a period of more than 5 years. As the total removal of the melanotic neurinomas is mandatory to prevent possible malignant transformation of the tumor recurrence or regrowth, we believe that the posterior and trans-thoracic approach are the most suitable one for the dorsal dumb-bell schwannoma.

     

Local excision of rectal schwannoma using transanal endoscopic microsurgery: A case report

INTRODUCTION

Schwannoma is a neoplasm originating from the neural crest cells (schwann cells) that form nerve sheaths. These tumors are thought to be benign with little risk of malignant transformation. They rarely affect the gastrointestinal tract, and primary rectal involvement is extremely rare. Until 2013, only 11 cases of anorectal schwannoma have been reported. Optimal surgical treatment of rectal schwannoma has not been established.

PRESENTATION OF CASE

We herein describe a 70-year-old woman with a submucosal tumor arising from the posterior wall of the rectum with features mimicking a gastrointestinal stromal tumor. After discussing the operative procedures and obtaining written informed consent, we attempted local excision of the tumor using a transanal endoscopic microsurgery (TEM). The tumor was proved to be S-100 positive schwannoma on immuhistochemical studies. Her postoperative course was uneventful, and there is no evidence of tumor recurrence as of 6 months after surgical excision.

DISCUSSION

An extremely rare rectal schwannoma was successfully treated using a TEM without compromising anorectal function.

CONCLUSION

TEM is a feasible approach for local excision of rectal tumors with low risk of malignancy.     

Adrenal Schwannoma Treated With Laparoscopic Surgery

Objective:

We report a case of left adrenal schwannoma in a 62-year-old man, incidentally discovered on an abdominal computed tomography. It was successfully treated with laparoscopic adrenalectomy.

Methods:

On admission, no remarkable findings were recognized in the patient''s blood and urine examination, including adrenal function. Laparoscopic left adrenalectomy was performed with the diagnosis of a nonfunctioning adrenal tumor.

Results:

Macroscopically, the tumor (45 mm × 30 mm, 60 g) arose from the medulla of the adrenal gland with a clear border distinguishing it from surrounding tissues. Histologically, the tumor consisted uniformly of spindle cells that were positive for S-100. The cortex was compressed but showed no atrophy. The diagnosis of adrenal schwannoma was made.

Conclusion:

Although an increasing number of adrenal incidentaloma have been identified with the recent advances in imaging techniques, only a few cases of schwannoma of the adrenal gland have been reported. We reviewed the cases reported previously in an attempt to reveal the characteristic features of this rare disease.     

Entrapment of the saphenous nerve at the adductor canal affecting the infrapatellar branch – a report on two cases

Objective:

To present 2 cases of entrapment of the saphenous nerve at the adductor canal affecting the infrapatellar branch, and to provide insight into the utilization of nerve tension testing for the diagnosis of nerve entrapments in a clinical setting.

Rationale:

Saphenous nerve entrapments are a very rare condition within today’s body of literature, and the diagnosis remains controversial.

Clinical Features:

Two cases of chronic knee pain that were unresponsive to previous treatment. The patients were diagnosed with an entrapment of the saphenous nerve at the adductor canal affecting the infrapatellar branch using nerve tension techniques along with a full clinical examination.

Intervention and Outcome:

Manual therapy and rehabilitation programs were initiated including soft tissue therapy, nerve gliding techniques and gait retraining which resulted in 90% improvement in one case and complete resolution of symptoms in the second.

Conclusion:

Nerve tension testing may prove to be an aid in the diagnosis of saphenous nerve entrapments within a clinical setting in order to decrease time to diagnosis and proper treatment.     

Gastric schwannoma: a benign tumour often mistaken clinically, radiologically and histopathologically for a gastrointestinal stromal tumour--a case series

INTRODUCTION

Gastric schwannomas are rare mesenchymal tumours that arise from the nerve plexus of the gut wall. They present with non-specific symptoms and are often detected incidentally. Pre-operative investigation is not pathognomonic and many are therefore diagnosed as gastrointestinal stromal tumours (GISTs). Operative resection is usually curative as they are almost always benign, underpinning the importance of differentiating them from GISTs.

METHODS

Three cases of gastric schwannomas were identified over a seven-year period. The clinical details and management were reviewed retrospectively.

RESULTS

There were two women and one man with a mean age of 62 years (range: 51–69 years). Two patients presented with bleeding and one with abdominal pain. The mean tumour size was 5.2cm (range: 2–10cm) and the tumours were resected completely following total or wedge gastrectomies. Histology in all cases showed spindle cells with a cuff of lymphoid tissue. Immunohistochemistry confirmed positive S100 staining and negative CD117 and DOG-1 staining in all cases.

CONCLUSIONS

We report our experience with these unusual primary stromal tumours of the gut and their presentations, preoperative investigations, operative findings and pathological findings are discussed. Operative resection in all cases has been considered curative, which is supported by previous series confirming the excellent prognosis of gastric schwannomas.     

The Tampa Scale of Kinesiophobia and neck pain, disability and range of motion: a narrative review of the literature

Background:

The Tampa Scale of Kinesiophobia (TSK) that was developed in 1990 is a 17 item scale originally developed to measure the fear of movement related to chronic lower back pain.

Objective:

To review the literature regarding TSK and neck pain, perceived disability and range of motion of the cervical spine.

Methods:

Medline, MANTIS, Index to Chiropractic Literature and CINAHL were searched.

Results:

A total of 16 related articles were found and divided into four categories: TSK and Neck Pain; TSK, Neck Pain and Disability; TSK, Neck Pain, Disability and Strength; and TSK, Neck Pain and Surface Electromyography.

Conclusion:

The fear avoidance model can be applied to neck pain sufferers and there is value from a psychometric perspective in using the TSK to assess kinesiophobia. Future research should investigate if, and to what extent, other measureable factors commonly associated with neck pain, such as decreased range of motion, correlate with kinesiophobia.     

Nerve sheath myxoma of the dorsal paravertebral space

INTRODUCTION

Nerve sheath myxomas (NSM) are rare benign soft tissue tumors. The dorsal paravertebral placed NMS diagnosis can be difficult.

PRESENTATION OF CASE

This article presents clinical, radiological findings and treatment of the NSM of the dorsal paravertebral space in a 32-year-old man presented with a right shoulder and back pain for 4 years.

DISCUSSION

NSM is a rare and benign tumor and that most often occurs in the skin of the head, neck or upper limbs of younger patients. Rare locations such as intracranial, spinal canal, trunk, lower limb and oral cavity were also reported. The appropriate treatment of NSM is surgical excision. Diagnosis is difficult in an uncommon presentation.

CONCLUSION

Although the most presented case of NMS are dermal tumors, it may also be found extremely rare locations. We conclude that, the definitive treatment of NSM is surgical excision with safe margins even when it is possible.     

Intraosseous neurilemmoma of the proximal ulna

INTRODUCTION

Neurilemmoma is a benign nerve sheath neoplasm commonly located in the soft tissue. Intraosseous neurilemmoma is rare, constituting less than 1% of primary bone tumors.

PRESENTATION OF CASE

A 21 year-old woman was presented with left elbow pain of 1-month duration. Plain radiographs showed a well-defined, lytic and expansile lesion of the proximal ulna. Computed tomography revealed cortical destruction and soft tissue extension. Because the tissue of origin for the tumor was uncertain, an open biopsy was performed. The specimens demonstrated a benign spindle cell tumor suggestive of a neurilemmoma, similar to a soft tissue neurilemmoma. The diagnosis of intraosseous neurilemmoma was established. Marginal excision of the soft tissue component and curettage of the lesion in the bone were performed. After 3.5 years of follow up, there is no clinical or radiographic finding to suggest any recurrence.

DISCUSSION

The major site of intraosseous neurilemmoma is the mandible. Occurrence in the long bone is particularly rare. Only two cases of intraosseous neurilemmoma involving the bones around the elbow have been reported to our knowledge; these cases arose in the distal humerus. We describe the first case of intraosseous neurilemmoma of the proximal ulna of the left elbow. The recommended treatment is conservative resection and bone grafting, as malignant change is extremely rare.

CONCLUSION

Although very rare, intraosseous neurilemmoma should be taken under consideration in the differential diagnosis of painful, radiographically benign-appearing osseous tumor around the elbow.     

The diagnosis and management of femoro-acetabular impingement

INTRODUCTION

Femoro-acetabular impingement (FAI) was first described in 1999 as abnormal abutment between the acetab-ulum and the femoral head and neck. Since then, it has been shown to be responsible for many acetabular labral tears and is implicated in the aetiology of osteoarthritis of the hip. This review introduces the concept of FAI and reports the key aspects of its diagnosis and management.

MATERIALS AND METHODS

A comprehensive search of the literature was conducted using the Pubmed database. Articles relating to the aetiology, pathophysiology, clinical features, diagnosis and treatment of FAI were reviewed. Search terms included femoro-acetabular impingement, arthroscopic treatment, open treatment, aetiology, pathophysiology. The search was limited to articles published in English. All articles were read in full by the authors and selected for inclusion based on relevance to the article.

RESULTS

An increasing number of studies relating to FAI have been produced in the 10 years since its recognition. A range of clinical and radiological features have been described. Surgical management can be performed using a number of techniques, with promising results from various studies. Early treatment with open surgery has paved the way for less invasive and arthroscopic approaches, with short-to-medium term data demonstrating favourable functional results for arthroscopic treatment of FAI.

CONCLUSIONS

A greater awareness of the diagnostic features of FAI, and the various management options available, will allow timely diagnosis and treatment of a relatively newly recognised syndrome. Early treatment may then help to prevent progression to end-stage osteoarthritis of the hip.     

A Rare Case of Anterior Chest Wall Schwannoma Masquerading as a Breast Tumor

A schwannoma is a tumor that develops on peripheral nerves or spinal roots. Although any part of the body can be affected, the breast is a quite unusual site for schwannomas. We report herein a case of schwannoma presenting as a breast tumor. In the current case, the tumor showed both clinically and mammographically as a well-defined breast mass. Of interest, sonographically, the well-defined mass appeared to be located in subcutaneous tissue, not in breast parenchyma, and this finding was confirmed histopathologically. These findings indicate the possibility that a schwannoma arising from subcutaneous breast tissue can show exophytic growth to the breast and appear as a breast tumor. In other words, our case implies the possible presence of a “pseudo” breast schwannoma.Key words: Schwannoma, Breast, Breast cancerA schwannoma is a relatively rare neoplasm that occurs from Schwann cells of the peripheral nerve sheath.^1–3 Although schwannomas may occur in any organ, a breast schwannoma is extremely rare and accounts only 2.6% of schwannomas.^1–4 We report herein a case of schwannoma suspected to be a breast tumor. The concern with breast schwannoma is a differential diagnosis, since on mammogram it sometimes resembles a breast cancer or tumor.     

Diagnosis and treatment of hepatic angiomyolipoma

Background

Hepatic angiomyolipoma (HAML) is a rare liver tumor. This paper summarized the clinical, radiological and pathological features of HAML.

Methods

Seventeen cases of HAML were analyzed retrospectively. All patients were subjected to surgical resection of tumor, one of which was performed emergency surgery because of hemorrhage of tumor.

Results

There are 13 females and 4 males, most of whom were asymptotic except 4 had minimal abdominal discomfort. US, CT and/or MRI were taken and corresponding data was comprehensively analyzed with other clinical signs and symptoms. Correct preoperative diagnosis was able to be achieved in 9 patients. Pathological analysis and immunohistochemistry of HMB-45 was used as final diagnosis. All patients were followed up and survived without recurrence.

Conclusions

Preoperative diagnosis of HAML can be benefited from comprehensive analysis of clinical manifestations. The malignant potential and fast growth of tumor suggested surgical removal of tumor while it was diagnosed.Key Words: Hepatic tumor, angiomyolipoma, diagnosis, surgery     

A rare etiology of acute abdominal syndrome in adults: Gastric volvulus – Cases series

INTRODUCTION

Gastric volvulus is a rare surgical emergency with a high mortality rate that requires urgent surgical management.

PRESENTATION OF CASE

A 19-year-old male and 51-year-old female patient underwent emergency surgery with a prediagnosis of acute abdomen syndrome, and a 60-year-old female patient underwent elective surgery due to diaphragmatic hernia. Abdominal exploration revealed gastric volvulus together with perforation in received emergency surgery patients, and a mesenteroaxial gastric volvulus due to diaphragmatic defect in third patient.

DISCUSSION

Gastric volvulus is classified into four subgroups depending on the mechanism of development, and organoaxial form is the most common type of gastric volvulus. The most challenging step in diagnosing gastric volvulus is the consideration of this diagnosis.

CONCLUSION

Preoperative diagnosis is often difficult, and its management involves surgical correction of the pathology followed by institution of resuscitative treatment.     

A giant adrenal cyst difficult to diagnose except by surgery

Introduction

Adrenal cysts represent rare clinical entities. Although surgical indications are well defined, pitfalls arise from the failure to establish an accurate preoperative diagnosis. Cystic lesions of other abdominal organs especially the pancreas complicate the diagnostic field.

Presentation of case

We present the case of a giant adrenal cyst in a young female causing diagnostic dilemma. Imaging studies revealed a large cystic lesion of uncertain origin located between the spleen and the tail of the pancreas. It was decided to perform a laparotomy which confirmed the presence of an adrenal cyst and enucleation of the cyst was performed. Examination at one year confirmed no complications.

Discussion

Adrenal cysts should always be included in the differential diagnosis of cystic abdominal lesions.

Conclusion

When the preoperative diagnosis is uncertain, surgical intervention can be both diagnostic and therapeutic.