Paratesticular rhabdomyosarcoma presenting as thickening of the tunica vaginalis

Pediatric paratesticular primary malignant tumors are rare, and most of them correspond to rhabdomyosarcomas (RMS). Paratesticular RMS has a better prognosis than RMS originating in other locations, because of its favorable histology and possible early diagnosis. The diagnosis relies upon the US findings of a paratesticular hypervascular mass in an adequate clinical setting. We report the unusual appearance of an infiltrating RMS tumor in a 3-year-old boy. The tumor presented as thickening of the tunica vaginalis. Atypical US appearance and discordance with clinical features caused suspicion of a tumoral process, allowing early diagnosis and treatment.     

Cardiac Teratoma of the Interventricular Septum with Congenital Aortic Stenosis in a Newborn

A 10-day-old infant presented with tachypnea, harsh murmur, and cardiomegaly. Echocardiography revealed a multicystic mass invading the entire interventricular septum and protruding into the left ventricular cavity without left ventricular tract outflow tract obstruction and aortic stenosis. The mass was subsequently resected and biopsy revealed it to be a cardiac teratoma. We present an unusually rare case of an intramyocardial teratoma with associated congenital aortic stenosis. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Paratesticular metastasis from Wilms tumor associated with a hydrocele

Metastatic sites other than the lungs, lymph nodes, and liver are unusual for Wilms tumor (WT). Intra-scrotal metastasis is very rare. We report a 3-year-old boy with stage IIA WT, who experienced paratesticular metastasis 2 months after surgery for an abdominal recurrence. He had right scrotal hydrocele at initial diagnosis. The patient underwent right radical orchiectomy, and pathological examination revealed paratesticular WT metastasis. Intra-abdominal and peritoneal disseminated metastases followed. We considered that tumor cells spread through the patent processus vaginalis and grew at paratesticular space in hydrocele. One month after the end of 12 months of salvage chemotherapy and abdominal radiotherapy, the patient has no evidence of disease.     

Teratoma in children under 7 years of age: analysis of 65 cases

This report concerns 65 children under 7 years of age treated during the last 10 years. Sacrococcygeal and ovarian teratomas were most common, but the incidence of retroperitoneal, cervical, and gastric teratomas was far greater than that published in other series (Table 1). Malignancy occurred in 18 cases (26%). Complete excision of benign tumors was curative although there was mortality due to the poor general condition of small patients with large tumors. Survival of the patients with malignant tumors was poor even after postoperative chemotherapy.     

Bilateral testicular tumors in an infant

A 7-month-old infant showed bilateral enlarged, nontender scrotal masses. The level of α-fetoprotein was greater than 10,000 ng/ml preoperatively; a high left inguinal orchiectomy was performed for a suspected yolk-sac tumor. The right testis was diagnosed as a mature teratoma because it was not possible to establish a line of cleavage between the tumor and the normal tissue, and a high right inguinal orchiectomy was performed. Only one case of bilateral testicular teratomas has been reported in the literature to date. We report a rare second case of bilateral testicular tumors, one a yolk-sac tumor and the other a teratoma. Accepted: 24 September 1996     

Intrapericardial teratoma

Summary An 8-month-old boy presented with respiratory stridor and recurring bronchitis due to an intrapericardial teratoma. The tumor was successfully removed. This is believed to be the first reported case of intrapericardial teratoma shown to contain insulin-producing cells.     

小儿卵巢肿瘤34例临床分析

目的：了解影响小儿卵巢肿瘤生存率的因素，探讨合理的治疗方案以提高治愈率。方法：３４例小儿卵巢肿瘤患儿为１９７３～１９９５年所收治，年龄４～１４岁，右侧１８例，左侧１６例，其中３例合并蒂扭转。均经手术及病理证实。良性２４例（７０．６％），恶性１０例（２９．４％）。结果：２９例获随访。２４例良性畸胎瘤术后１例复发；２例恶性畸胎瘤术后无瘤生存期分别为６年和２年；３例内胚窦瘤术后均死于肿瘤广泛转移。结论：对于良性卵巢肿瘤主张行患侧附件切除。对于Ⅰ、Ⅱ期恶性肿瘤除行患侧附件切除外，应剖视对侧卵巢，并进行活检、术后化疗及严密随访。对Ⅲ、Ⅳ期恶性卵巢肿瘤强调作根治性手术，尽可能切除原发灶及所有转移病灶，同时术后化疗，以达长期缓解。     

Neonatal Intracerebral Teratoma

A neonate, who presented with a large head but no neurological abnormalities, was diagnosed by ultrasound and CT scan as having a brain tumor. The pathological diagnosis was teratoma with matured tissues. The literature is briefly reviewed and the clinical manifestations and histological classification are documented.     

Ultrasound features of a paratesticular arteriovenous malformation: a case report of an 11-year-old boy

A paratesticular arteriovenous malformation (AVM) in an 11-year-old boy with a solitary testicle led to a testicle-sparing excision. The radiological features of this rare lesion are reviewed.     

Paratesticular desmoplastic small round cell tumors: A case report and review of the literature

Desmoplastic small round cell tumor (DSCRT) is a rare malignancy most often seen in the abdomen or pelvis of young men. Unfortunately, this disease is usually metastatic at diagnosis and has dismal outcomes. We describe a case of isolated paratesticular DSCRT in a 14‐year‐old male successfully treated with surgical resection, chemotherapy, and adjuvant radiation, and we present a review of the relevant literature. It appears that isolated, paratesticular DSCRTs have a markedly better outcome than the classic abdominal or pelvic location. We hypothesize that this is due to earlier detection and the relative ease of surgical resection.     

Pilomatricoma in childhood: a retrospective study from three European paediatric centres

Pilomatricoma is characterised as a common, slowly growing benign cutaneous tumour that appears generally within the first decades of life. The clinical diagnosis is frequently missed, especially by the paediatrician unfamiliar with these tumours. We present the experience gained in three European tertiary care paediatric centres with the treatment of pilomatricoma and also current data on the aetiology, clinical presentation and management. A retrospective study was carried out in 83 patients suspected for pilomatricoma during a 7-year period (1996–2002) at the departments of Paediatric Surgery of the Childrens University Hospital Federico II, Naples, Hospital San Bortolo, Vicenza and Aghia Sophia Childrens Hospital, Athens. The age range was from 10 months to 17 years, median age 8 years. All patients were treated by surgical excision and all specimens were examined by histopathological assessment. The follow-up varied from 5 months to 6 years. The correct diagnosis was made preoperatively in 68 patients (82%). The female/male ratio was 2:1. The sites of occurrence were the head (47.5%), especially in the periorbital region, the neck (9%), the upper limbs (35.5%), the inferior limbs (4%) and the thorax (4%). Each patient exhibited a single pilomatricoma except for two patients who had multiple lesions (2.4%). One of them had Steinert disease (myotonic dystrophy). No recurrences were observed during the follow-up period. Conclusion:Pilomatricoma is one of the most common cutaneous adnexal neoplasms in children. Surgical excision including clear margins and its overlying skin in most cases is the treatment of choice. The recurrence as well as malignant evolution is rare.     

Fetus in fetu: a case report

A fetus in fetu is defined as a condition where a parasitic twin is found included within the body of its partner [8]. This is a report of a fetus in fetu treated successfully in a 4-mounth-old boy that fulfilled all the criteria of the disease. A brief review of the literature is also included.     

Long-term functional results following resection of neonatal sacrococcygeal teratoma

Purpose  Sacrococcygeal teratoma (SCT) is the most common congenital neoplasm in neonates. We wished to assess the long-term functional outcome of children undergoing SCT resection. Methods  Records of neonates diagnosed with SCT from two surgeons’ practices, and operated on between 1970 and 2006, were retrospectively reviewed. Patients/parents who consented to participate in the study received a questionnaire, focusing on fecal and urinary continence, constipation and lower extremity weakness. Results  Forty-six patients were identified. Four had died (3 from malignant tumors and 1 motor vehicle accident at 18 years). Of the 42 remaining cases, 39 were benign and 3 were malignant; 2 of the former developed malignant recurrences. Twenty-seven agreed to participate and 14 (52%) completed the questionnaire. Median age of respondents was 16.7 years (3–29), and none of the respondents had a recurrent tumor. Thirteen of the 14 respondents experienced no problem with urinary or fecal incontinence, or lower extremity weakness. The remaining patient had all three problems, but his SCT had involved the spinal cord. Of the ten patients who commented on constipation, one had significant constipation, five occasional constipation, and four no constipation. Conclusions  Functional results after resection of neonatal SCT are excellent, with only a small number of patients reporting problems with fecal or urinary continence, or lower extremity weakness. Constipation is relatively common. This information is important for counseling families with fetal or neonatal SCT.     

Teratoma of the tongue in neonates: report of a case and review of the literature

A female newborn presented with a huge mass protruding from the mouth. She had no respiratory distress but had difficulty swallowing. The mass originated from the tongue and was completely extirpated on the day of birth. The histologic diagnosis was mature teratoma. Three days later, another mass measuring 1 cm in diameter was found attached to the upper pharyngeal wall and was also completely extirpated 20 days after birth. The histologic diagnosis was also mature teratoma. The infant had a complete cleft palate, but no other malformation was found. Two years after surgery there is no sign of recurrence. Accepted: 22 August 1997     

Intracardiac teratoma in an infant

Summary We report the only infant known to us who has survived surgery for a primary right ventricular teratoma. Included is a review of other reported cases of teratoma of the heart in infancy and childhood.     

Teratoma in a thyroglossal duct cyst: A rare occurrence

Although teratoma is a relatively commonly encountered pediatric tumor, cervical teratomas are rare, with only 217 cases recorded to date. These are thought to arise from the thyroid gland, with only anecdotal reports of teratomas arising in thyroglossal duct cysts. Correspondence to: G. E. Besner M. D.     

Acquired pulmonary stenosis: Ultrasonographic diagnosis

A 15-year-old boy presented with chest pain and a new heart murmur. The clinical diagnosis of pulmonary stenosis was confirmed by two-dimensional and Doppler echocardiographic examinations. A large, encapsulated, partly solid and partly cystic tumor in the anterior mediastinum, visualized by ultrasonography, was compressing the main pulmonary artery and producing the right ventricular outflow tract obstruction. The tumor was removed surgically and was found to be a benign teratoma. Postoperative ultrasound examination of the right ventricular outflow tract showed no evidence of obstruction. We conclude that: (1) there are subtle clinical diagnostic differences between intrinsic and extrinsic pulmonary stenosis; (2) ultrasound examination can make cardiac catheterization unnecessary; and (3) relief of the extrinsic cause of pulmonary stenosis should be curative.     

Mediastinal teratoma in a neonate

A neonate with severe respiratory distress due to a benign mediastinal teratoma (MT) is reported. Despite early and easy surgical excision of the tumor, the child died due to poor cardiac function. Only ten cases of MT in neonates have been reported in the literature so far. While the tumor has been known to interfere with lung development in utero, postnatal myocardial dysfunction due to poor heart development has not been previously documented. Accepted: 22 September 1997