Jaroslav Kraus

Cystic lymphangioma of the neck in adults: a report of three cases

ZusammenfassungLymphangiome sind seltene Läsionen der lymphatischen Kanäle, die oft bereits bei der Geburt bestehen und zumeist (in 90% der Fälle) vor dem 2. Lebensjahr diagnostiziert werden. Nur wenige Fälle eines Auftretens im Erwachsenenalter wurden bisher in der Literatur beschrieben. Die Therapie der Wahl ist die komplette operative Entfernung. Manchmal ist dies allerdings nicht möglich, da der Tumor dazu tendiert, sich entlang lebensnotwendiger Strukturen auszubreiten. Wir beschreiben die klinischen und pathologischen Eigenschaften von zystischen Lymphangiomen bei 3 Erwachsenen: Die Tumoren traten alle im lateralen Halsbereich auf. Es wurden eine klinische Untersuchung, Ultraschall, MR sowie FNAC durchgeführt. Wir diskutieren die Schwierigkeiten bei der korrekten Diagnose dieser seltenen Läsion, die in vielen Fällen erst bei der histopathologischen Untersuchung möglich ist Die therapeutischen Optionen werden ebenfalls kurz beschrieben. Unsere Fälle zeigen, dass bei der Differentialdiagnose lateraler Halstumoren zystische Lymphangiome berücksichtigt werden sollten. Außerdem betonen sie die Notwendigkeit für eine adäquate Bildgebung und Zytologie, um die präoperative Verdachtsdiagnose zu erhärten.SummaryLymphangiomas are uncommon lesions of lymphatic channels that are often present at birth and diagnosed mostly (90%) before the age of two years. Lymphangiomas occur exceedingly rarely in adults and few cases are described in the literature. The treatment of choice is complete surgical removal; however, the tumor tends to spread along vital structures therefore sometimes inductive complete surgical removal is impossible. We describe the clinical and pathological features of cystic lymphangioma diagnosed in three adults with lateral neck mass. Clinical examination, ultrasonography, MRI and fine-needle aspiration cytology were performed. We discuss the diagnostic difficulties of this uncommon lesion where in many cases the correct diagnosis is reached only after histopathological investigation of the surgical specimen. Therapeutic options are briefly described. Our cases suggest the need to consider cystic lymphangioma in the differential diagnosis of lateral neck masses in adults, together with adequate imaging and cytological studies to corroborate the preoperative diagnosis.

Coincidence of thyroid tumor and thyroglossal duct remnants. Review of the literature and presentation of three cases

Aims and Background The coincidence of benign or malignant thyroid tumors with thyroglossal duct remnant (TDR) cysts is rare. Although the precise etiology is still unclear, thyroid origin and spread from a primary site have been suggested and this obviously has important implications for the therapeutic approach. Three cases of thyroglossal duct carcinoma are presented and its management is discussed on the basis of the current rationale for treatment of thyroid cancer. The indication for surgery depends on positive findings in the thyroid gland (nodules, FNAB). The aim of this study was to review our experience in the management of papillary thyroid diseases associated with TDR. Materials and Methods The records of three patients with thyroid tumors associated with TDR treated at the Department of ENT and Head and Neck Surgery of the First Medical Faculty UK of Prague between January 1991 and January 2001 were analyzed. We searched for risk factors of thyroid carcinoma: history of ionizing radiation, history of thyroid diseases, age, tumor size, tumor spread and histopathological factors. Results We used a triple approach consisting of clinical and ultrasound examination and fine-needle aspiration biopsy for preoperative assessment. Our diagnostic and therapeutic procedures included TDR excision (Sistrunk or Schlange procedure) and total thyroidectomy. Although the therapeutic approach could be a matter of discussion, most patients agreed with our suggestion of relatively radical but non-mutilating treatment. Postoperative radiation or radioiodine ablation is considered in cases of TDR carcinoma or thyroid carcinoma associated with TDR. Oncological follow-up included clinical and ultrasound examination three times during the first year, twice in the second year, and once yearly thereafter. Tumor marker evaluation and/or scintigraphy were performed 6, 12 and/or 24 months following surgery.

Prevalence of Helicobacter pylori in adenotonsillar hypertrophy in children

Abstract Conclusion: Our results encourage the notion that the pharynx could be an extragastric reservoir of Helicobacter pylori (HP). The study confirmed the presence of HP in adenotonsillar tissue in children. It could have importance in the pathophysiology of upper respiratory diseases. However, its precise role in these processes remains unclear and requires further studies. Objective: A prospective study was carried out to evaluate the presence of HP in tonsillar and adenoid tissue in children. The study focused on real-time PCR analyzing CagA and VacA genotypes of HP strains. Methods: A total of 37 consecutive pediatric patients with adenotonsillar hypertrophy indicated for surgery were observed in a prospective study. Adenoidectomy and/or tonsillectomy was performed in each patient; 49 specimens were taken, 32 from adenoids and 17 from tonsils. The presence of HP and its genotype were tested in all samples by real-time PCR analysis. Results: Of 49 samples analyzed, 48 were positive for the presence of HP (98%), so only 1 sample was negative. While the genotype VacAs1bm2 was definitely dominant in adenoid tissue, wider distribution was observed in tonsillar tissue. Cag+ strains represented one-fifth of all samples (21%).

Giant angiofibromas in tuberous sclerosis complex: a possible role for localized lymphedema in their pathogenesis.

BACKGROUND Giant angiofibromas in patients with tuberous sclerosis complex (TSC) are rare. OBJECTIVE We sought to report two patients who had TSC with unusually large and disfiguring facial angiofibromas and to identify underlying histopathologic changes that may possibly explain the clinical features. METHODS We performed a clinicopathologic, immunohistochemical, and molecular biologic study using 42 lesional specimens and peripheral blood from one of the two patients. The immunohistochemical investigations were mainly focused on the vascular moiety of the lesions. TSC1 and TSC2 alterations were studied using multiplex ligation-dependent probe amplification for large deletion/duplication mutations, whereas screening for small mutations was performed using polymerase chain reaction amplification of individual coding exons and exon-intron junctions of both genes followed by an analysis on denaturation gradient gel electrophoresis. RESULTS Histopathologic examination revealed, in addition to findings typical of angiofibroma, several unusual features including multinucleated giant cells containing multiple intracytoplasmic vacuoles, Touton-like cells, emperipolesis, pagetoid dyskeratosis, vacuolar alteration at the dermoepidermal junction, Civatte bodies, and melanophages in the subjacent dermis. Numerous dilated lymphatic vessels were detected indicating localized lymphostasis, probably caused by secondary lymphedema. The lymphatic nature of the vessels was confirmed by immunohistochemical study. Genetic testing for TSC1 and TSC2 gene mutations revealed a substitution on position c.2251C>T resulting in a nonsense mutation R751X in fragment 20.2. LIMITATIONS Histopathologic specimens and peripheral blood were available from only one patient. CONCLUSION Localized lymphedema may contribute to the formation of large disfiguring angiofibromas in patients with TSC.