Plexiforme neurofibroma with compromising of the penile function

The objective of this article was to describe a case of Von Recklinghausen's disease with plexiform neurofibroma of the penis. The patient was 26 years old and reported difficulty with sexual intercourse owing to a penile mass, despite experiencing normal sensation and erection. Physical examination demonstrated this penile mass with café au lait spots, subcutaneous neurofibromas, and lisch nodules. The treatment consisted of subcoronal incision and resection of the lesion. Pathologic examination revealed a plexiform neurofibroma. The patient recovered well with subsequent maintenance of penile sensation and erection. In cases of plexiform neurofibroma compromising penile function, total resection of penile mass offers a good therapeutic option.     

Hypertrophy of clitoral hood: presenting sign of neurofibromatosis in female child

Although clitoral involvement with neurofibromatosis is rare, all cases previously reported have described clitoral hypertrophy due to neurofibromas of the clitoral corpora. We report on a patient who had localized enlargement of the prepuce only, with no evidence of neurofibromatous infiltration. In all cases, diagnosis of clitoromegaly requires basic chromosomal and endocrinologic evaluation. However, recognition of its association with neurofibromatosis due to either neurofibromas of the genitalia or localized genital hypertrophy may spare the patient an unnecessary or invasive evaluation. Because of an association with urinary tract neurofibromas, the patient with genital involvement should have cystoscopy. Clitoroplasty with sparing of the neurovascular bundle and glans is the preferred method of management of the enlarged clitoris.     

Genitourinary neurofibromatosis in a child presenting with an enlarged penis and scrotum

We report on a boy with genitourinary neurofibromatosis. The patient presented with bilateral hydronephrosis, and a markedly enlarged penis and scrotum. Laparotomy showed a mass occupying the small pelvic cavity. Histologically, the mass was a plexiform neurofibroma. The enlarged genitalia seemed to be caused by involvement of neurofibromatosis. The patient underwent a successful ileal conduit diversion.     

Benign neural sheath tumours of major nerves: Characteristics in 119 surgical cases

Summary Peripheral benign nerve sheath tumours are infrequent tumours and affect major nerve trunks. Some authors have indicated a high and prohibitive incidence of neurological injury in resection of these lesions. The authors describe their findings in a retrospective study comprising 119 patients with spontaneous benign nerve sheath tumours of the peripheral nervous system. Seventy-three patients had a schwannoma, 41 had neurofibroma and 5 had plexiform neurofibroma; 25 of the 119 patients suffered from neurofibromatosis. All schwannomas were excised completely and the outcome of patients was 41.0% improved, 6.8% worsened, 52.0% unchanged. Twenty-eight neurofibromas were excised completely and 13 subtotally; the outcome for patients was 19.5% improved, 19.5% worsened and 61% unchanged. All plexiform neurofibromas were removed subtotally and the outcome for patients was 20% improved and 80% unchanged. The best surgical results at average follow-up of 6 years were observed in the patients with schwannoma, the worst in those with plexiform neurofibroma. Our results demonstrated that it is often possible to remove schwannomas as well as neurofibromas with an acceptable risk of injury to the nerve.     

Benign nerve sheath tumors on urinary bladder biopsy

Benign nerve sheath tumors (schwannoma and neurofibroma) involving the urinary bladders are rare with only case reports and limited series. We identified 6 neurofibromas and 2 schwannomas involving the urinary bladder. Of the 8 cases, 7 were sent to one of the authors in consultation and the last came for treatment to The Johns Hopkins Hospital. Patients (3 males, 5 females) ranged in age from 3 to 69 years old (average 36 y old). Presenting symptoms included recurrent infection in 6 patients, hematuria in 3 patients, and irritative symptoms in 3 patients (some patients with 2 symptoms). Three patients had a pelvic mass on physical examination. Three patients (3/8) had a solitary lesion in the bladder, 4 patients (4/8) had multifocal lesions, and 1 patient had no information available. Five patients underwent local resection. One patient, who was 3 years old, had multiple plexiform neurofibromas within the bladder and did not have surgical treatment and another patient is also undergoing surveillance. One patient had no treatment information available. Seven patients had clinical follow-up information available. The length of follow-up varied from 2 to 124 months (average 47 mo). Three out of the seven patients with follow-up information had neurofibromas in other sites, including the skin, uterus, mesentery, and ureter. One patient was known to have other stigmata of neurofibromatosis. Both cases with schwannoma had only bladder involvement. No family history of neurofibromatosis and no genetic studies were performed on any of the patients. Three neurofibromas were of the diffuse type, with 2 cases plexiform, and 1 case could not be subtyped. Neither recurrence nor malignant transformation was demonstrated on follow-up. The importance of accurately diagnosing plexiform neurofibromas of the bladder is that a subset harbors neurofibromatosis. If the lesion is focal, conservative excision is the choice of treatment with a low risk of recurrence. Diffuse neurofibromas can be difficult to diagnose leading to delay of treatment and potentially the need for a more extensive excision. Once recognized as a neurofibroma, it is important to identify it as a diffuse neurofibroma, given its lack of relationship to neurofibromatosis.     

Large Solitary Plexiform Neurofibroma of the Penis Without Erectile Dysfunction: a Case Report from Kerala

Case Report

Solitary plexiform neurofibroma of the penis is an extremely rare peripheral nerve lesion with only about 20 cases reported all around the world. Here, we are reporting a case of solitary penile plexiform neurofibroma with sexual dysfunction due to difficulty in penetration. This 47-year-old gentleman presented with a swelling on the dorsal side of the penis. He was aware of the swelling for the last 15 years. The swelling was slow growing and there was no associated loss of sensation or sexual dysfunction. For the last 2 months, he noticed that the tumour is rapidly increasing in size. He was also having difficulty in penetration during sexual intercourse which forced him to seek medical attention. He underwent excision of the swelling from our department. After 18 months of follow-up following excision, there has been no evidence of recurrence of the tumour.

Conclusion

Penile plexiform neurofibromas are a rare type of lesions and are usually associated with neurofibromatosis. Solitary penile plexiform neurofibromas are extremely rare. Excision, when done properly, gives good results, without erectile dysfunction.

     

A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve

Plexiform neurofibroma is generally considered as a component of neurofibromatosis 1, and a great majority of the cases with plexiform neurofibroma display other symptoms related to neurofibromatosis. Plexiform neurofibromas occur frequently in the head and neck region due to the rich innervation of the area, however appear rarely in the extremities. We report here an isolated giant plexiform neurofibroma involving the common peroneal nerve branches without symptoms related to neurofibromatosis in a 5-year-old case. Surgical excision was performed due to pain and numbness in the leg, and against the possibility of malignant transformation due to sudden growth observed in the tumor. One month following the excision of all the reachable tumoral tissues, tendon transfer surgery was performed for the ankle and toe extensions. Our case stands as the only reported case of isolated giant plexiform neurofibroma involving the common peroneal nerve in the pediatric age.     

Neurofibroma of the clitoris. A case report

We report an unusual case of clitoral hypertrophy due to neurofibromatosis of the external genitalia. Because of no definite skin signs or other nervous or osseous symptoms such as are commonly found in neurofibromatosis, the patient was at first suspected of being a case of hermaphroditism. Finally, the disorder was properly diagnosed after laparotomy followed by clitorectomy. This patient seems to be the 12th case of clitoral neurofibroma reported in the English literature.     

Mesenteric plexiform neurofibroma in an 11-year-old boy with von Recklinghausen disease

We experienced the case of an 11-year-old boy diagnosed as having type 1 neurofibromatosis with intraabdominal and pelvic masses. On physical examination, there were multiple café-au-lait spots larger than 15 mm in diameter scattered over the patient's entire body and axillary freckling, but no cutaneous neurofibromas were present. Lisch nodules were detected in the iris by a slit lamp. A large, firm mobile mass was palpated in the lower abdomen. Abdominal computed tomographic scan showed the hypodense masses in the lower abdomen and pelvic cavity. At laparotomy, a 16 × 9-cm, firm nodular mass along the mesentery of the terminal ileum and ascending colon was found. The mesenteric mass was encasing the superior mesenteric vessels and extending into the serosa of the intestine. The mass was incompletely excised together with affected intestine. However, resection of the rectum could not be performed because of the extensive involvement of the entire mesorectum. Histopathologic study revealed a plexiform neurofibroma involving the mesentery and intestine without evidence of malignant transformation. The postoperative course was uneventful. Ten months after the operation, a magnetic resonance imaging showed no interval change of the mesorectal mass.     

Utilization of skin flap for reconstruction of the genitalia after an electric burn

The objective of this article is to describe a case of an electric burn to the genitalia causing scrotal and testicular lesion, and the subsequent reconstruction using a skin graft. The patient was a 10-year-old boy who was victim of an electric burn that harmed the genitalia. There was extended skin loss, penile, scrotal and partial testicular lesion. The treatment consisted of plastic surgery to reconstruct the genitalia with skin flaps grafted on the left thigh, the scrotum and the base of the penis. The patient recovered well and was discharged after two weeks. We concluded that in severe cases of electric burns to the genitalia, skin graft offer a good therapeutic option.     

Neurofibromas of the bladder in a child with neurofibromatosis type 1 causing chronic renal disease

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal-dominant inherited disorder and its prominent feature is the neurofibroma and renal involvement includes renal artery stenosis and renal artery aneurysms causing renovascular hypertension. The genitourinary tract may be rarely involved, leading to urinary symptoms of obstruction and hydronephrosis. Herein, we report a 12-year-old boy with chronic renal failure associated with neurofibromas of the bladder, which leads to urinary obstruction.     

Misdiagnosis of plexiform neurofibroma of the medial plantar nerve: Case report

Plexiform neurofibromas are benign tumors of the peripheral nerve. Diagnosis may be challenging, if they present mimicking other peripheral nerve pathologies.We report the case of a patient who had severe foot pain, which progressively hampered her walking ability, erroneously attributed to recurrent Morton's neuroma. Diagnosis of plexiform neurofibroma of her right medial plantar nerve was made 15 years after the appearance of symptoms.Pain and function recovered after radical neurotomy of the medial plantar nerve. A correct diagnosis is an essential starting point in the treatment of neurofibromas and a misdiagnosis may lead to an inappropriate treatment.     

A case report: surgical reconstruction of the external genitalia in adrenogenital syndrome

This is a case report of a 5-year-old girl with congenital adrenal hyperplasia (CAH) who had clitoral hypertrophy and vagina opening at verumontanum between the bladder neck and external urethral sphincter. Female pseudohermaphroditism with CAH is the most common type of intersex problem seen in children. In females with this disorder, the internal genital organs are usually normal, but variable degrees of virilization can be observed externally. We discussed the indication of the surgical correction of ambiguous external genitalia in this syndrome in view of sexual function and cosmetic problems.     

Manipulation of postnatal testosterone levels affects phallic and clitoral development in infant rhesus monkeys

Male primates exhibit marked elevation of circulating testosterone levels during the early postnatal period. The aim of this project was to test whether experimental manipulation of circulating testosterone levels in male and female infant rhesus monkeys affected development of the external genitalia during the first six months of life. Four groups of infants were studied. Seven control male infants exhibited high circulating testosterone levels during the first three months of life. Seven males were treated with a GnRH agonist (avorelin) from the first week of life onwards, which suppressed the postnatal testosterone surge. Ten control females exhibited low circulating testosterone levels during the early postnatal period. Administration of testosterone to 10 females resulted in high circulating levels in these infants. Fortnightly blood samples and genital measurements were taken from all infants during the first six months of life. Growth of the penis of avorelin-treated males was significantly retarded when compared to control males. Average length of the penis at six months of age was significantly (p = 0.012) smaller for avorelin-treated males (25.2 +/- 2.8 mm) than for control males (37.3 +/- 3.0 mm). Avorelin-treated males attained only around 50% detachment of the prepuce from the glans of the penis, while control males averaged 90% detachment. Treatment of females with testosterone resulted in significant growth of the clitoris in comparison to control females. The growth rates of the penis of control males and clitorides of testosterone-treated females were similar and greatest during the first two months of life. Gain in body weight was not affected by either hormonal manipulation. It is concluded that manipulation of circulating testosterone levels during the early postnatal period affects penile and clitoral development of infant rhesus monkeys. This postnatal period may therefore represent an important stage in penile development in primates.     

Penile duplication

We report on a case of penile duplication in an eight-year-old boy. While diphallia was incomplete, there was a common root of the penis which divided into two shafts of different size, doubling in frontal plane. Only one urethra left the bladder along the lower and smaller penile shaft and ended with a hypospadiac meatus. After surgical plastic repair, the patient had a good functional and cosmetic result. In addition, this patient had a horseshoe kidney.     

Genitourinary neurofibromatosis in childhood

Neurofibromatosis in children occurs uncommonly and involvement of the bladder is exceedingly rare. The syndrome is transmitted as an autosomal dominant trait and is characterized by cutaneous pigmentation and tumors of neural crest origin. Hypertension has been documented in association with neurofibromatosis in the form of renovascular disease in children and with pheochromocytomas in adults. We report on 2 children with extensive neurofibromas of the bladder, upper tract damage and genital enlargement. One case was misdiagnosed as posterior urethral valves and the other case as clitoral hypertrophy. The literature of urogenital neurofibromatosis is reviewed with particular attention to bladder lesions, hypertension, malignant potential and treatment options.     

Plexiform Neurofibroma of the Submandibular Salivary Gland: A Rare Tumour

A 15 year old boy presented with swelling in the submandibular region. X ray of the part showed faint radio opaque shadow. A provisional diagnosis of sialadenitis with sialiolithiasis was made. Excised mass was reported histopathologically as plexiform neurofibroma of submandibular salivary gland. Plexiform neurofibroma of the salivary gland is a rare benign tumour, often present in the parotid gland. It is very rare in submandibular salivary gland. It is a slow growing, locally infiltrating tumour.     

Temporary claviculectomy approach for plexiform neurofibroma of the first intercostal nerve

Plexiform neurofibroma at the thoracic inlet has rarely been reported and to our knowledge, the use of a temporary middle claviculectomy approach for thoracic inlet tumors has never been reported. We report a case of plexiform neurofibroma of the first intercostal nerve resected using a temporary claviculectomy approach. An abnormal shadow detected radiographically in a 16-year-old boy led to a diagnosis of neurofibromatosis 1 (NF-1) with a chest wall tumor at the thoracic inlet. The patient underwent resection of the tumor with the right first rib. The resected clavicle was reapproximated with a plate and postoperative shoulder function was satisfactory. The tumor was diagnosed pathologically as a plexiform neurofibroma and the patient’s postoperative course was uneventful. The temporary middle claviculectomy approach provides excellent exposure of the subclavian vessels and brachial plexus before resection of the tumor. We recommend this approach for tumors of the anterior thoracic inlet.     

Acute arterial insufficiency of the male genitalia

Three patients developed severe ischemia of the penis or scrotum from acute arterial occlusion. In one case, nonhealing ulceration of the glans developed after atheroembolism to the dorsal penile artery. One patient had penile ischemia after ligation of pelvic and femoral collateral circulation during repair of an aorto-bilateral-iliac artery aneurysm. A third patient had ischemia of the penis and scrotum from thromboembolism to the iliac arteries during repair of an aortoenteric fistula. Only seven patients have been described with acute arterial occlusion and severe ischemia of the male genitalia. A rare phenomenon because of rich collateral circulation, acute ischemia of the genitalia nevertheless must be recognized as a sign of severe vascular disease and a consequence of major arterial ligation or occlusion in the pelvis and groins.     

阴茎缺损的治疗进展

阴茎为男性的重要外生殖器官,主要具有排尿、排精和性交功能。战伤、创伤、外伤以及某些疾病如阴茎癌均可造成阴茎完全或部分缺损,严重导致尿流改道和丧失性交能力,给患者精神和生活上造成巨大的创伤。因此阴茎缺损的修复日益受到患者和医生的高度重视,理想的治疗应达到具有良好的外形和感觉、通畅的站立排尿、顺利完成性交3个方面的和谐统一。目前阴茎缺损临床治疗主要采用阴茎再植术、阴茎延长术及阴茎再造术;随着移植免疫、组织配型、免疫抑制剂的研究进展,以及动物异体肢体移植的长期存活等令人鼓舞的结果,为人体异体阴茎移植治疗阴茎缺损奠定了坚实的基础;组织工程技术构建阴茎组织绝大部分尚处于实验阶段,如何在临床上将构建的组织与自体组织有效的融合,在重建阴茎外观的基础上,使之发挥持久的功能将是一项值得继续研究的课题。