Fibroepithelial polyp of the prostatic urethra in an adolescent

Benign fibroepithelial polyp of the prostatic urethra is a rare congenital lesion most commonly diagnosed in the pediatric population because of diverse urinary symptoms. We report a case of a 16-year-old adolescent boy who presented with urinary retention. Imaging and endoscopic studies confirmed the presence of a polypoid lesion at the bladder base/posterior prostatic urethra. Following transurethral resection of this polypoid mass, the diagnosis of a congenital fibroepithelial polyp of the prostatic urethra was rendered by pathologic evaluation. Although this is considered a benign lesion with no previous reports of recurrence or malignant behavior, it produces dramatic urinary symptoms in the pediatric population with a wide differential diagnosis. Imaging and endoscopic findings may suggest a malignancy and are not sufficient to render a precise diagnosis, which can only be made by pathologic examination.     

A solitary bone cyst in the spinous process of the cervical spine: a case report

STUDY DESIGN: A case report. OBJECTIVES: To illustrate a rare case of histologically confirmed solitary bone cyst involving the spinous process of C7. SUMMARY OF BACKGROUND DATA: A solitary bone cyst involving the spine is very unusual. Although four cases of a solitary bone cyst in the spine have been reported in the literature, the current authors have been able to find only one case of solitary bone cyst in the spinous process. All four patients reported in the literature were over 30 years of age. The patient in the current case was a 13-year-old girl with no history of trauma. METHODS: Radiographs and a computed tomography scan of the cervical spine were performed before the operation, as was a histologic examination to make a diagnosis of the lesion. RESULTS: The intraoperative findings from examination of the stagnant fluid within the lesion and the histologic examination indicated the diagnosis of a solitary bone cyst. CONCLUSIONS: A solitary bone cyst in the spine is rare, especially in the young. An osteolytic lesion in the spinous process of the spine tends to be diagnosed as an osteoblastoma or as a giant cell tumor of the bone. A solitary bone cyst of the spine, although rare, must be considered as a differential diagnosis.     

A Cerebriform Mass on the Right Buttock

BACKGROUND: Nevus lipomatosus cutaneous superficialis of Hoffmann-Zurhelle (NLCS) is a rare disorder that is characterized by tumors of mature fat cells situated within the dermis. OBJECTIVE: To illustrate by a case report the clinical presentation and management of this rare condition. METHODS: Wide excision of the right thigh and buttock mass was performed, and pathological material was submitted. The defect was repaired with a split-thickness skin graft. RESULTS: The largest measured nevus lipomatosus, congenital type (20 x 30 cm), was removed from this patient. CONCLUSION: Physicians should be aware of this rare tumor, which can become extremely large if untreated.     

Oral Congenital Melanocytic Nevus: A Rare Case Report and Review of the Literature

Melanocytic nevi are congenital or acquired benign proliferations of cells of melanocytic origin. Oral congenital melanocytic nevi are rare, and only a few cases have been reported in the literature. The purpose of this study is to present the clinical, histological and immunohistochemical features of an oral congenital melanocytic nevus in a 16-year-old female with an 11-year follow-up and to review the pertinent literature. The reported case is the fifth well-documented case report of oral congenital melanocytic nevus in the English literature and the first with a long period of follow-up, thereby making it an important contribution to the knowledge regarding this uncommon oral mucosa lesion.     

Bilateral congenital pseudarthrosis of the clavicle report of a case with clinical, radiological and neurophysiological evaluation.

Congenital pseudarthrosis of the clavicle is a rare condition. Bilateral involvement is extremely rare: only seven cases have been reported in the literature. Although the anatomy of the thoracic outlet can be markedly altered by the hypermobility of the shoulder and although few cases with mild symptoms suggesting brachial plexus impairment have been reported, the neurological status of the brachial plexus in congenital pseudarthrosis of the clavicle has not been well assessed. We report a case of bilateral congenital pseudarthrosis of the clavicle in which clinical, neurophysiological and radiological evaluations were performed.     

Intradural extramedullary mature cystic teratoma: not only a childhood disease

OBJECTIVE: The authors report on the first case of a spinal intradural extramedullary cystic teratoma in an aged patient. These lesions have been reported in adolescents and young adults often with a history of spinal dysraphism. They are believed to be congenital lesions; however, they have also been reported in patients with a history of posterior spinal surgery or lumbar puncture. METHOD: An 85-year-old man was evaluated for persistent and progressive lower extremity paresis. His symptoms began after relatively minor trauma. He had no prior lumbar surgeries. Neuroimaging studies revealed an L1-L2 intradural mass. A putative diagnosis of a cystic intradural extramedullary mass was made. A lumbar laminectomy and durotomy were performed. RESULTS: A cystic lesion containing hair follicles, cartilage, adipose, and neural tissue was encountered. Pathologic review corroborated the diagnosis of cystic teratoma. CONCLUSIONS: The occurrence of cystic teratomas in the absence of previous surgery or lumbar puncture is uncommon. Even rarer are reports of these lesions in aged patients. Of particular interest in this case is the fact that this patient had not undergone any previous lumbar procedures nor did he have a history of spinal dysraphism. Though rare, this entity should be included in the differential of cystic intradural spinal cord lesions.     

Ligament reconstruction in congenital absence of the anterior cruciate ligament: a case report

Congenital absence of the cruciate ligament is an extremely rare condition that was first reported in Giorgi's radiographic study in 1956. The authors report on a case of anterior cruciate ligament reconstruction performed on a 21-year-old female patient with congenital anterior cruciate ligament absence. We also discuss radiographic evidence that could provide clues to the congenital absence and possible difficulties that may be encountered during surgery with a review of the relevant literature.     

Female epispadias: are we missing the diagnosis?

OBJECTIVE: To describe three of four female patients with occult epispadias, as usually epispadias is reported to be a rare condition in females (1/480,000), and obvious and easy to diagnose, but less severe variants may still involve urethral sphincter incompetence in the absence of obvious clinical signs. PATIENTS AND METHODS: The hospital records over a 10-year period (1 January 1991 to 31 December 2000) were reviewed to find all female patients diagnosed with epispadias. Data were collected from the Consultative Council on Obstetric and Paediatric Mortality and Morbidity for the number of live births per year for the decade studied, and the incidence of female epispadias determined. RESULTS: Over the 10-year period there were 13 patients with a diagnosis of epispadias, from 636,698 total live births; four were female and nine male. Each of the female patients had varying degrees of epispadias; three had subtle signs with apparently normal external genitalia, and only one had a bifid clitoris. However, even the most minor variant case had a palpable notch in the symphysis pubis, and an 'oblong' external urethral meatus. Thus the incidence of female epispadias is at least 1/160,000. CONCLUSION: Epispadias in females may be up to three times more common than previously expected, but even experienced clinicians can miss the diagnosis. The importance of a careful examination should be emphasized, especially in female patients with chronic wetting of unknown cause. A bifid clitoris is an important sign but not always present. Key diagnostic features include drug-resistant wetting, a very low leak pressure on cystometrography, a palpable gap in the pubic symphysis and an abnormal oval-shaped external urethral meatus.     

Vaginal and phallic urethra with prominent clitoris in female pseudohermaphroditism

Idiopathic female pseudohermaphroditism associated with a vaginal urethra, prominent clitoris and an accessory phallic urethra is a rare syndrome, with no apparent cause to account for the masculinization of the genitalia and exhibit of similar characteristic features. We report such a case and review the pertinent literature.     

Congenital pouch colon with rectal atresia: a case report

The association of congenital pouch colon with rectal atresia is quite rare with only 2 cases previously reported in literature. We describe the third such case and the second instance to survive. Although the prior survivor was managed by a single-stage procedure, we successfully managed our case by staged procedures. In this case report, we discuss the etiology and surgical options available for this rare condition.     

阴蒂异常勃起1例报告并文献复习

目的 :探讨阴蒂异常勃起的临床特点。　方法 :报告 1例 2 9岁已婚女性患者阴蒂异常勃起 ,结合文献复习阴蒂异常勃起的病因、诊断和处理方法。　结果 :患者阴蒂异常勃起 6 0h ,经在阴蒂海绵体内注射α肾上腺素激动剂后 ,阴蒂异常勃起治愈。　结论 :阴蒂异常勃起极其罕见 ,其原因主要是使用抗抑郁药物和盆腔恶性肿瘤压迫和浸润阴蒂静脉使血液回流受阻所致 ,阴蒂海绵体内注射α肾上腺素激动剂是一种行之有效的治疗方法。     

Palmar Basal Cell Carcinoma: Case Report and Literature Review

BACKGROUND: Although basal cell carcinoma (BCC) is the most common tumor of the skin, its occurrence on the palm is very rare. Only eight cases not associated with the basal cell nevus syndrome having been reported in the world literature so far. Among these eight cases, one was associated with epidermolysis bullosa dystrophica and another with previous trauma to the site of the appearance of the lesion. OBJECTIVE: The authors report a case of palmar BCC due to its rare occurrence on that site. The case is not associated with any predisposing factors such as basal cell nevus syndrome, trauma, or preexistent dermatosis. METHODS: An incisional biopsy was performed into the border of the palmar ulcer. The histopathologic findings of the fragment, stained by hematoxylin and eosin, were characteristic of BCC. RESULTS: Following surgical removal of the lesion, there was complete healing and the patient has presented no signs of relapse after 3 years of clinical follow-up. CONCLUSION: Palmar BCC not associated with the basal cell nevus syndrome or any other predisposing condition is very rare. Nevertheless, when facing an ulcerated lesion on the palm, BCC should be considered as a diagnostic possibility.     

Rare Malignant Tumors of Clitoris—a Case Report

Tumors of the clitoris are very rare. Isolated case reports are available in literature. This case report highlights the importance of this disease, due to its high malignant potential and treatment options. We had come across a rare tumor of the clitoris in a young girl, reported to be malignant extrarenal rhabdoid tumor and patient had a short life span of 6 months.     

Diffuse pancreatic adenocarcinoma identified in an adult with annular pancreas

Annular pancreas is a congenital anomaly resulting from malrotation of the pancreatic ventral bud. Although annular pancreas in the adult is rare, it may be recognized with increased frequency as a result of more liberal use of pancreatic imaging studies in patients with chronic abdominal pain and suspected chronic pancreatitis. Malignancy in the setting of annular pancreas is an uncommon event that has been reported previously but has almost always been related to the annular (ventral) segment. We report an interesting case in which pancreatic adenocarcinoma diffusely involving the dorsal (nonannular) segment presented in a middle-aged female patient. This unusual presentation points out the importance of considering neoplasia as part of the differential diagnosis and the possibility of pancreatic pathology in the dorsal, nonannular segment when there is no obvious duodenal or biliary obstruction involving the annular ventral segment.     

Sequelae of an undiagnosed unilateral congenital fixed anterior shoulder dislocation in a 40-year-old female

Congenital shoulder dislocation is a rare condition. A review of the literature showed that 0.018–0.07% of newborns suffer from shoulder dislocations. There have been only few reports about this entity, clinic and radiologic features, treatment, and etiology of this condition remains unclear. We report a case of undiagnosed unilateral congenital fixed anterior shoulder dislocation in a 40-year-old female, which affected the day quality of life. Pediatrists and orthopaedic surgeons should know this rare condition and the importance of a proper diagnosis in the early childhood when an appropriate treatment could be performed.     

Cystic lymphatic malformation of bladder presenting as a pelvic mass

We report a 5-year-old boy with a cystic lymphatic malformation (LM) of bladder, and the imaging characteristics of the lesion are reported. Cystic LM of the urinary system is rare, and a location in the bladder is extremely rare. The exact mechanism of the lesion that occurs in bladder is unknown. This case is different from the 3 cases reported before, for it is only presented with a palpable pelvic mass and the lesion did not penetrate full thickness of bladder. Although imaging examinations are helpful in the definition of cystic LMs, no characteristic findings are available to diagnose a cystic LM of the bladder before surgery. The diagnosis is always made after surgical intervention. Surgical excision is ideal, and an extensive operation is not warranted because the lesion is benign. Once excised completely, the prognosis of the disease is good.     

Urachal tuberculosis

BACKGROUND: We report on an extremely rare case of urachal tuberculosis that was confirmed using a polymerase chain reaction test of paraffin-embedded material. METHODS/RESULTS: A 62-year-old man presented with pollakiuria. With a diagnosis of urachal abscess, the patient underwent en bloc resection of the cystic mass. A bacterial culture test of the content showed no organism. The histopathologic findings suggested urachal tuberculosis. The AMPLICOR polymerase chain reaction test by using paraffin-embedded sections revealed the existence of Mycobacterium tuberculosis in the resected tissue. The only positive finding in systemic screening examinations for tuberculosis was old tuberculosis scars in the upper right lung. It was supposed that hematogeneous spreading from the lung lesion may result in urachal tuberculosis after a long latent period. CONCLUSIONS: Although urachal tuberculosis is an extremely rare condition, tuberculosis must always be kept in mind when observing any infectious diseases.     

Heterotopic pancreatic tissue in the gallbladder. Two case reports and brief review of the literature

Heterotopic pancreas tissue in the gallbladder is a rare benign condition with no clinical relevance and found incidentally in most cases. Only 28 cases of heterotopic pancreas are reported until 2007 in the worldwide literature. The preoperative diagnosis is very hard, so that it could be considered in differential diagnosis with other benign and malignant disease of gallbladder and biliary tree. The surgical approach for the excision of a undefined lesion and the histological identification is a correct procedure in suspect case. We report 2 cases of heterotopic pancreatic tissue of the gallbladder.     

Osteoid osteoma in the base of the coracoid process of the scapula. Excision by anterior approach: a case report.

Osteoid osteoma in the base of the coracoid process of the scapula is very rare and diagnosis and treatment often is delayed. A lesion in this atypical location may seem surgically unreachable. This report is of a case of osteoid osteoma in the base of coracoid process in a 14-year-old female. The lesion had been diagnosed as a nontumorous condition and overlooked for four years. Computed tomography and magnetic resonance imaging revealed a nidus in the base of the coracoid process. The en bloc excision of the osteoid osteoma was managed by an anterior approach using an osteotomy of the coracoid process. A 12-month follow-up examination revealed no symptoms and computed tomography showed bone healing with no recurrence of the tumor.     

Report of a case of sinonasal undifferentiated carcinoma arising in a background of extensive nasal gliomatosis

BACKGROUND: Sinonasal undifferentiated carcinoma (SNUC) is a relatively rare, aggressive malignancy of adulthood. Nasal glial heterotopia is a benign congenital condition in which mature benign brain tissue develops outside the central nervous system. METHODS: A 37-year-old man was seen with right nasal obstruction, epistaxis, and headache. Imaging showed a radiologically malignant mass lesion. Biopsy and subsequent resection were performed in the evaluation and treatment of this lesion. Histologic and immunohistochemical assessments of the biopsy and resection specimens on the pathology material classified the lesional tissue. RESULTS: Histology and immunohistochemistry of the biopsy specimen demonstrated an SNUC that was subsequently resected following chemotherapy. In addition to SNUC, the resection specimen revealed extensive, multifocal nasal glial heterotopia (so called "nasal glioma"). CONCLUSIONS: This is the first known case in the literature in which these 2 relatively rare entities coincide.