Symptomatic Periungual Fibroma of the Hand

Periungual fibromas are benign nodules commonly found on acral digital areas that are commonly associated with tuberous sclerosis. They vary in size and are challenging to treat, with a high recurrence rate. We present a case of a patient with a periungual fibroma, which by virtue of its size, was of functional concern. The intraoperative findings and their implications on the clinical outcome, together with a literature review on other treatment modalities, are also presented.     

Multidisciplinary treatment of patients with gender dysphoria

Summary Patients with gender dysphoria are best treated by a multidisciplinary team. Such a team consists of a nucleus of specialists who sees the patient frequently and a peripheral group that sees patients incidentally. The tasks of each team member are described, and the value of the multidisciplinary cooperation is highlighted. Gender dysphoria patients are better treated with a team approach than with treatment by individual specialists who are working independently of one another.     

Rare tumors of the lateral ventricle. Review of the literature

Metastases of lateral ventricle (LV) are attached to choroidal plexus. Primary source is cancer of kidney. Two thirds of patients are male. Oligodendrogliomas occur in young females. Signs of increased intracranial pressure are a constant feature. These tumors are found in anterior portion of the LV with severe enhancement and clumped calcifications. Treatment is surgical. Cavernomas have a preponderance of rapid growth with a bleeding revelation. Seizures are rare. Rebleeding is frequent and justifies a surgical treatment. Schwannomas are a rare entity in which the majority of patients are very young. MRI shows calcifications, cystic components and a strong enhancement. They are limited to sporadic cases, never associated with neurofibromatosis. Arachnoid cysts are located in the atrium and/or in the occipital horn. Patients are young (mean age < 40 years). MRI demonstrates an intracystic lesion with signal intensity similar to the CSF. Best treatment is an endoscopic fenestration. Epidermoid cysts occur in third decade. These pearly tumors appear isointense or a little hyperintense on T1-weighted imaging, very characteristic. They are enhanced after gadolinium injection and appear strongly hyperintense on T2-weighted imaging. An incomplete removal with a thorough long-term follow-up is necessary. Cavernomas of LV are hyperintense on T1- and T2-weighted imaging. They have a bleeding risk of 25 to 45%. Therefore, they must be operated.     

Mediastinal germ cell tumors

Mediastinal germ cell tumors are uncommon tumors that occur predominantly within the anterior mediastinum and frequently present as a very large mass with local compression. Symptoms are typically vague and represent the local mass effects of the tumor. Chest computed tomography and examination of serum tumor markers provide the critical workup before a tissue diagnosis is obtained. Seminomas are extremely sensitive to both chemotherapy and radiation and are primarily treated nonsurgically. Benign teratomas without malignant elements are extremely resistant to both chemotherapy and radiation and are treated exclusively with surgical resection with excellent outcomes. Malignant nonseminomatous germ cell tumors are primarily treated with chemotherapy, with adjuvant surgery reserved for patients with residual mediastinal masses after systemic therapy.     

Klinefelter's syndrome and precocious puberty: a harbinger for tumor

Boys with Klinefelter's syndrome are at an increased risk of precocious puberty. Most cases are either idiopathic or due to a mediastinal tumor. Patients with Klinefelter's syndrome are at a high risk of primary, extragonadal germ cell tumors, which are usually nonseminomatous, but can be a mixed type with seminomatous elements. The differential diagnosis of precocious puberty includes mediastinal tumors, especially in boys with Klinefelter's syndrome.     

Endovascular treatment of penetrating injury of axillary vein with Viabahn endoprosthesis

Penetrating injuries of the axillary or subclavian venous system are associated with extensive blood loss and are fatal in more than 50% of cases. Patients are usually unstable and are treated with surgical exploration. We present a case of axillary venous injury that was treated in the operating room with intravenous placement of a self-expanding Viabahn endoprosthesis (W.L. Gore). The device was delivered to the injured site percutaneously via a basilica vein, with immediate control of hemorrhage.     

Uric acid, phosphate and oxalate stones: treatment and prophylaxis

Medical treatment for the most commonly encountered types of renal stones is described. Nowadays treatment for uric acid stones is well-defined: alkalinizing urine is easy with drugs that are sufficiently active and well enough managed. Relapse is avoided in a high percentage of patients. Medical treatment of phosphate or calcium stones is a more open question as results are far from satisfactory compared with intra- and extra-corporeal approaches which are often minimally invasive and well accepted by both patient and urologist. Relapses are not easy to control because prophylactic measures such as changes in lifestyle and diet are never activated or because they are adopted for a brief period of time. Water therapy is examined, with the choice of water depending on the type of stone, together with drug therapy. Drugs such as citrate, with or without magnesium, and thiazides are considered excellent for curing renal stones and relapses. Although medical therapy has a limited role in many types of stones, its use is decisive in some others.     

Lower lumbar hyperextension fracture without paraplegia

Lumbar shear injuries are a rare injury with few cases documented in the literature. They are the result of hyperextension combined with anteroposterior shearing forces. Those with near-complete displacement are usually associated with significant neurologic deficit. Presented here is a case of L4-L5 fracture-dislocation with no neurologic loss.     

Lunate fractures in the face of a perilunate injury: an uncommon and easily missed injury pattern

Lunate fractures are rare and are usually associated with high-energy trauma. Typically, they are described in isolation or with associated carpal injuries such as scaphoid, capitate, or radial styloid fractures. We report a case of a complex lunate fracture in combination with a perilunate dissociation injury.     

Recurrent bradycardia and delayed recovery in a neonate following repair of nasofrontal encephalocoele with holoprosencephaly and single cerebral ventricle

Holoprosencephaly and single cerebral ventricle are uncommon congenital anomalies that are associated with a high rate of perinatal mortality. We describe a neonate who developed recurrent bradycardia along with delayed recovery following frontal craniotomy for the repair of a nasofrontal encephalocoele associated with holoprosencephaly and a single cerebral ventricle. The neonate, however, recovered following elective ventilation. Etiopathogenesis and management of such complications are discussed.     

Thoracic myelocystoceles – two variants

Summary Myelocystoceles are rare lesions. Rarer still are thoracic myelocystoceles. Two patients with thoracic myelocystoceles are being reported. The first patient presented with swelling in the thoracic region with paraplegia and incontinence. MR with MR myelography revealed the malformation to be a thoracic myelocystocele. The second patient presented with a midline cutaneous mass lesion without neurological deficit. MRI revealed a Type I split cord malformation, a CSF containing sac in the thoracic region that communicated through a stalk-like structure to the hydromyelic hemicord. Surgery in both these patients did not produce any change in the neurological status. The importance of recognizing thoracic myelocystoceles, their clinical presentation, embryology, value of MR myelography in the diagnosis and role of surgery are discussed. The differences between terminal myelocystoceles and thoracic myelocystoceles are also highlighted.     

Infectious endocarditis of a Chiari network

We present the case of a 62-year-old man with infectious endocarditis in a Chiari network. Chiari networks are present in 1.5% to 3% of the population. Although Chiari networks are usually clinically insignificant, they are associated with a number of conditions, including patent foramen ovale, thromboembolism, atrial aneurysm, and cardiac arrhythmias. Although there are rare reports of patients with a Chiari network who had endocarditis develop, this is the first report of a patient who had endocarditis develop solely within a Chiari network.     

Gastric hamartoma of the pylorus in an infant

Gastric hamartomata, alternatively called adenomyomata, are rare, benign lesions of the gastric wall. They can present with a wide spectrum of symptomatology. Pure pyloric adenomyomata are exceedingly rare. We are reporting a 13-day-old infant with a gastric hamartoma at the pylorus presenting with gastric outlet obstruction mimicking infantile hypertrophic pyloric stenosis.     

A presentation of glandular penile metastases from prostate adenocarcinoma

Secondary tumours of the penis are rare; they most commonly arise from the prostate and the bladder. These lesions are often associated with disseminated malignancy and have a poor prognosis, with a 6-month mortality of up to 80% reported. Penile metastases have a variety of clinical manifestations including incidental penile nodules, cutaneous findings, urinary symptoms, pain and malignant priapism. Treatment options are mainly targeted at improving the patients' quality of life and are tailored to their clinical condition, but are primarily palliative. This study reports a case of a 92-year-old man with a presentation of glandular penile metastases from prostate adenocarcinoma treated conservatively.     

Coexisting thymic carcinoid tumor and thymoma

Thymic carcinoid tumors are unusual neoplasms that are different from thymomas. We report a case of coexisting thymic carcinoid tumor and thymoma associated with myasthenia gravis. The clinicopathological findings are discussed with a review of the literature.     

Myasthenia gravis in children and its anaesthetic implications

Myasthenia gravis, a rare disease in children, occurs in a variety of forms. The aetiology and clinical presentations are reviewed. Eight patients who were studied with electromyography are presented. The results show that, in general, patients with the usual pattern of disease are resistant to suxamethonium (ED95 3-4 times normal) and are sensitive to nondepolarizing relaxants. When the latter are used it is advisable to administer small increments with neuromuscular monitoring. One patient with the disease localised to the eyelid had normal EMG responses when monitored on the hand with ulnar nerve stimulation.     

Clinical outcomes after treatment of germ cell tumors

Intracranial GCTs are a heterogeneous group of neoplasms most commonly diagnosed in the pediatric population. Germinomas are exquisitely radiosensitive with long-term survival rates in excess of 90% with radiotherapy alone. NGGCTs are associated with a poorer prognosis and are typically treated with a combination of radiation and chemotherapy. Given the young age of these patients, achieving optimal outcomes will ultimately require a careful balance of maximizing disease control while minimizing adverse treatment effects. Here we review the management of intracranial GCTs and discuss the clinical outcomes of patients who undergo treatment for these rare and fascinating tumors.     

Spleen-Restricted Posttransplant Lymphoproliferative Disorder in the First Year After Kidney Transplant – A Case Report

Posttransplant lymphoproliferative disorders (PTLDs) are a feared complication after transplant. They are mostly of B cell origin and are frequently Epstein-Barr virus (EBV)–positive, particularly in early onset PTLD. Later on, non-B and EBV-negative PTLD are increasingly reported. EBV seronegative receptors (particularly when paired with an EBV seropositive donor) together with the net degree of immunosuppression—a concept often difficult to quantify—are the most consistently described risk factors for the development of PTLD. Conversely, its association with a particular immunosuppressive agent or other virus, namely cytomegalovirus (CMV) infection or disease, has been inconsistently reported. We present a challenging case where an EBV negative monomorphic peripheric T-cell lymphoma was diagnosed in the first year after kidney transplant in a patient with a recent history of CMV disease from a resistant strain.     

Molecular organization of the plasma membrane in the post-acrosomal region of some farm animals

Summary. According to the distribution of IMP, three different regions can be recognized on PF of the post-acrosomal plasma membrane of bull, ram, and boar spermatozoa. They are: (1) a region with linear aggregation of IMP , (2) a region with fewer and scattered IMP , and (3) a region with more numerous IMP. In the last two regions IMPs are randomly distributed or a clustering of certain particles is visible. In stallion spermatozoa the last two areas are undistinguishible.
There are evident interspecies differences in the arrangement of linear aggregations of IMP which are characteristic for each species.     

Primary adenocarcinoma of the epididymis: case report

Primary epididymal malignancies are uncommon and usually benign. Benign paratesticular tumors are most commonly adenomatoid, while the most common malignant paratesticular tumors are rhabdomyosarcomas. Approximately 25% of all epididymal tumors are malignant, and of the benign tumors, 60% to 78% are adenomatoid tumors. According to a recent MEDLINE search using epididymis and adenocarcinoma as key words, reports of a primary epididymal adenocarcinoma are extremely rare with only 23 cases in the literature. We report a case of epididymal adenocarcinoma with clinical follow up and metastatic natural history of this rare malignancy.