Ralf-Bodo Tröbs

Oral tumors and tumor-like lesions in infants and children.

The aim of this retrospective study was to survey the spectrum of oral tumors and tumor-like lesions treated in a pediatric surgical unit. The clinical features and treatment outcome are presented, and guidelines for management discussed. Long-term follow-up was carried out both by re-examination and by means of a questionnaire. A total of 95 patients were encountered over a 30-year period. The age at presentation ranged from 1 day to 16 years, and the male to female ratio was 0.7:1. The lesions were located predominantly on the lips (22%), tongue (21%), and cheek (19%). Patients were divided into five groups based on histological diagnosis. Benign lesions accounted for 83 (87%) of the cases. Of these, 41 (43%) were benign tumors, the most common of which were the hemangiomas (17 cases). Hamartomas accounted for a further 22 benign lesions (23%), among which 12 were lymphangiomas. Furthermore, we saw 14 cases (15%) of mucoceles, ranula and dysontogenetic cysts, and a further 6 cases (6%) were classed as miscellaneous lesions. Simple surgical resection was successful in treating most benign lesions, with occasional re-excision being necessary in lymphangiomas. The long-term effects of treatment include reduction of the red volume of the lips, scarring following resection of parotid hemangiomas, a forked tongue after wedged resection, and partial facial nerve palsy. The group of 12 (13%) malignant tumors consisted of 5 rhabdomyosarcomas, 2 fibrosarcomas, 2 carcinomas of the parotid, 1 osteosarcoma, and 2 metastases. A multimodal approach was used in patients with rhabdomyosarcomas, while fibrosarcomas and parotid carcinomas were normally treated by surgical excision. Six of 12 patients with malignant tumors were alive after a median follow-up of 20.5 years. Re-examination of the malignant tumor group revealed scarring, impaired growth and function of the maxilla associated with local irradiation, and an external salivary fistula. In conclusion, while most oral and maxillofacial tumors of children are benign, malignant tumors of soft tissue, salivary glands and bones must be taken into account. There are specific aspects related to certain developmental and biological characteristics that make a mainly conservative approach preferable in these children.

Changing pattern of osteomyelitis in infants and children.

Abstract A retrospective analysis of 332 children with osteomyelitis (OM), managed from 1966 to 1996, was undertaken to evaluate etiology, clinical course and treatment results. In 64% of all patients positive bacterial cultures were obtained, Staphylococcus aureus, streptococci, pneumococci, and Haemophilus influenzae were the most frequently cultured pathogens. In two-thirds of the cases long bones (femur, tibia, humerus) were affected. Osteoarthritis or suppurative arthritis was evident in 27%; 32 of 170 (19%) re-evaluated patients had moderate or severe sequelae. Risk factors for an unfavorable course were the onset of disease in early infancy, suppurative arthritis, and an affected epiphysis. Suppurative arthritis, in particular, needs early evacuation to prevent sequelae. In recent years we observed an increasing number of patients presenting with atypical forms of OM. Since 1989 10 patients were considered to have chronic recurrent multifocal OM (CRMO). In 6 of them the clavicle was involved; their ages ranged from 3 to 14 years. The erythrocyte sedimentation rate was elevated (median 48, range 9–110 mm), while other inflammatory parameters like C-reactive protein (median 9, range <5–85 mg/l) or leucocyte count were slightly elevated or normal. Histopathology was stage-dependent, with a predominance of lymphoplasmacellular infiltration. A nonbacterial origin of CRMO is probable but not proven. Histopathology is not suitable for differentiation between bacterial and nonbacterial forms of bone inflammation.

Fibrosarcoma in infants and children: a retrospective analysis - overdiagnosis in earlier years.

Abstract During a 30-year period, 22 patients considered to have a fibrosarcoma (FS) were treated. In a retrospective study the clinicopathologic findings were summarized. With histologic and immunohistochemical re-evaluation, the diagnosis was confirmed in 8 cases. For 6 further patients FS was very probable but specimens were not available. In 8 cases the diagnosis was revised and benign lesions were found in 7. Two patients with irresectable tumors died (infantile FS, FS of mesentery and retroperitoneum). After repeated local recurrences and spread on the affected extremity, an amputation was life-saving in 1 boy. In earlier years many tumors were classified as FSs. Today, immunohistochemistry and molecular-biological methods are valuable tools to clearly identify these tumors. Wide local excision or en-bloc resection without sacrificing any significant function of the part should be the primary form of treatment in infants. Primary re-excision after incomplete excision should have priority over any adjuvant treatment. Preoperative chemotherapy may avoid incomplete resection or mutilation in cases with extended congenital FS.

Central lactic acidosis, hyperventilation, and respiratory alkalosis: leading clinical features in a 3-year-old boy with malignant meningeal melanoma

Meningeal tumors are extremely rare in children and are diagnostically as well as therapeutically challenging. Among the least common types of malignancies in childhood is malignant melanoma, counting for less than 1% of pediatric tumors. Due to the rarity and the wide spectrum of appearance, initial clinical features may be misleading. A 3-year-old boy was referred to our hospital with symptoms of hyperventilation, dyspnoea, tachycardia, respiratory alkalosis, inarticulate speech, and fatigue. Measurement of pH in cerebrospinal fluid (CSF) yielded central lactic acidosis despite alkalosis in peripheral blood. Diagnostic imaging procedures as well as histology and immunohistochemistry revealed the diagnosis of a malignant meningeal melanoma. We hypothesize that central lactate production of the tumor nests might have induced central acidification, thus inducing hyperventilation by stimulation of central chemoreceptors. This case is a model example of the key role of central pH as an inducer/suppressor of ventilation in humans and illustrates the critical importance of central pH for regulating both ventilation and acid-base homeostasis. Thus, pH of CSF should be measured whenever a malignant brain tumor is suspected.

Recurrent polytopic chromaffin paragangliomas in a 9-year-old boy resulting from a novel germline mutation in the von Hippel-Lindau gene.

Pheochromocytomas are frequently associated with inherited cancer syndromes such as von Hippel-Lindau disease (VHL). Retinal angioma and hemangioblastomas of the central nervous system are hallmarks of VHL, but its clinical variety is remarkably broad. Pheochromocytomas as the sole or first manifestation of VHL are rare but have been observed. In this case report, the authors describe an unusual case of initial collapse, seizures, and hypertensive crisis in a child who later was found to have multiple extraadrenal pheochromocytomas. Molecular diagnostics revealed a novel point mutation in the VHL gene (VHL nt. 406 T-->G). Only 7 months after the first lesions had been removed, a new paraganglioma developed in the contralateral periadrenal region. When encountering pheochromocytomas in children, the clinician should be aware that an associated tumor syndrome might be present, and appropriate molecular screening should be initiated. Molecular genetics aid in the clinical decision-making and clinical management of individual patients with pheochromocytoma.

Perineal ectopic testis: a rare cause of empty scrotum.

Perineal ectopic testis is a rare congenital anomaly and remains a urologic curiosity. We report the course of 2 recurrent cases after hormonal therapy and a third case associated with inguinal hernia. During the surgical exploration, the gubernaculum testis was found fixed to the perineum. The testes were placed in the scrotum. The evaluation of differential diagnosis of empty scrotum is recommended.

Surgical Anatomy of Large Retroperitoneal Teratomas in Infants: Report of Two Cases

We report on two infants of 11 and 12 months of age, respectively, with large solid-cystic retroperitoneal tumors. Complete resection was achieved, and both children are doing well over a follow-up of more than 17 months. The presented paper focuses on surgical anatomy of this very rare type of tumor. Teratomas were located mainly within the upper abdominal cavity, and both tumors displaced the pancreas in an anterior position. The following anatomical features were observed (1) displacement of surrounding organs, (2) deformation and elongation of large retroperitoneal vessels, (3) fibrous incorporation of large vessels by the tumor pseudocapsule, (4) wrapping of anterior aortic branches by lobes and fingers of the tumor.

Pheochromocytoma and multifocal functioning paraganglioma in a 9-year-old boy with von Hippel-Lindau disease.

We report the case of a 9-year-old boy presenting with multifocal paraganglioma partially involving the right suprarenal gland, abdominal paraganglia, and the left para-aortal retropleural region. Von Hippel-Lindau disease (VHL) was diagnosed exclusively by VHL-gene analysis. Other clinical signs of VHL were lacking. After a complaint-free period of 7 months the boy developed a metachronous paraganglioma adjacent to the opposite adrenal gland. Patients with VHL disease should undergo adrenal-sparing surgery.

Papillomavirus-induced genital warts in a girl--management by surgery and immunomodulating therapy.

Abstract A 4-year-old girl with condylomata acuminata of the vulva and papular warts of the surrounding skin is presented. The lesions were removed by surgery. Histologic investigation showed koilocytosis of the squamous epithelium and in-situ hybridization revealed human papilloma virus type 6 infection. There were no signs of sexual abuse or sexual transmission of the virus. After ablation, an interferon-containing ointment was applied. In order to prevent recurrence, a low-molecular-weight immunomodulating leucocyte fraction was given for more than 1 year, during which time no relapse was observed.

Treatment of transient posttraumatic bile-duct stenosis by laparoscopic-assisted cholecystotomy

Abstract.A 10-year-old boy developed severe obstructive jaundice following blunt abdominal trauma. Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed a stricture of the common bile duct. A cholecystostomy tube was inserted under laparoscopic guidance. After temporary bile drainage and a cholecystoenteric bypass the patient recovered.