Evaluation of Children and Adolescents with Thyroid Nodules: A Single Center Experience

Objective: We aimed to evaluate the clinical, radiological and pathological findings of children and adolescents with thyroid nodules. Methods: Data of 121 children and adolescent with thyroid nodules and had fine needle aspiration (FNA) were examined retrospectively. Concomitant thyroid disease, ultrasonography (US) features of the nodule, FNA and histopathological results were recorded. FNA results were assessed according to The Bethesda System for Reporting Thyroid Cytopathology (TBSRTC). Results: Median (range) age of the cases was 14 (3-18) years and 81% were female. FNA results of patients were: insufficient in 1 (0.8%); benign in 68 (56.2%); indeterminate in 44 (36.4%); and malignant in 8 (6.6%) patients. Among 39 patients who underwent surgery, 10 (25.6%) had differentiated thyroid cancer (DTC) and the overall malignancy rate was 10.0% (10/100). Follow-up FNA results showed progress based on TBSRTC in 18.7% of benign results and 4/75 patients had DTC on surgical excision. Two of 22 patients with atypia of undetermined significance (AUS) who continued follow-up was diagnosed with DTC. Male gender, presence of Hashimoto thyroiditis and US findings of uninodularity, hypoechogenicity, increased blood flow, irregular margins, solid structure, microcalcification and presence of abnormal cervical lymph nodes were associated with malignancy. Conclusion: In this study 10% of thyroid nodules were malignant in children and adolescents. Patients with AUS have a 9% potential for malignancy. Patients with initially benign FNA result may have changes on repeat FNA when assessed with TBSTRC indicating a 5.3% false negative rate.

Sentinel nubbin: A potential pitfall in the management of undescended testis secondary to epididymo-testicular nonunion.

INTRODUCTION: Epididymal anomalies are common in boys with undescended testis. Epididymo-testicular non-union with a nubbin in the scrotum is a rare anomaly and may be confusing during management of cryptorchidism. The non-fused epididymis and vas deferens which are descended to the scrotum, may be regarded as nubbin tissue secondary to testicular atrophy and proximal testis may be overlooked. OBJECTIVE: To evaluate a relatively rare anomaly of epididymo-testicular non-union in which vas deferens and epididymis are descended to the scrotum resembling a nubbin tissue, while a separate testis remains in a proximal peritoneal fold (hernia or processus vaginalis)which could potentially be missed during the management of patients with undescended testis. STUDY DESIGN: The charts of the fourteen patients with epididymo-testicular non-union and a scrotal nubbin are retrospectively evaluated. RESULTS: Median age of the patiens was 1.5 years (0.5-12), 11 were left and 3 were right. No connection could be demonstrated between the testis and vaso-epididymal unit in any of the patients. Orchidopexy was performed in 13 patients while orchidectomy in1 patient. The scrotal nubbin tissue is excised in 4 patients and histological examination revealed sections of vas deferens and epididymis. One of the patients underwent inguinal exploration and orchidectomy for assumed testicular atrophy elsewhere but histological examination revealed vas deferens and epididymis without any sign suggesting an atrophic testis. An intrabdominal testis was found incidentally in this patient and he underwent 2 additional procedures for orchidopexy. DISCUSSION: Although epididymo-testicular anomalies are common in boys with undescended testis, nonunion is the rarest type of this anomaly. Similar cases are reported in the literature as simple fusion anomalies with indefinite clinical significance or even polyorchidism although no accessory testis could be demonstrated. However, the scrotal nubbin tissue which actually contains epididymis and vas is a potential decoy and may result in missing of the proximal testis if regarded as a sign of testicular atrophy and further evaluation is obviated. As this is retrospective study, actual incidence of the condition is yet to be determined. CONCLUSION: A scrotal nubbin tissue may be secondary to epididymo-testicular nonunion as well as testicular atrophy. As feasibility of radiological imaging studies are limited, a laparoscopic exploration may be considered to confirm or to exclude a separate testis in patients with scrotal nubbin. Prospective studies are needed to evaluate the actual incidence of the condition and gain-loss analysis of laparoscopic exploration.

Clinicopathological Assessment of Kidney Biopsies in Children with Familial Mediterranean Fever: A Single-Center Experience.

OBJECTIVES: Familial Mediterranean fever (FMF) is a monogenic auto-inflammatory disease which might rarely cause glomerulopathy in patients. The aim of this study was to determine the clinical, demographic, and genetic characteristics and type of glomerular lesions in pediatric FMF patients who underwent kidney biopsy. METHODS: The data of 30 pediatric FMF patients with biopsy-proven glomerulopathy were retrospectively reviewed. Patients were grouped into 2 categories as amyloid nephropathy (AN, n = 16) and non-amyloid nephropathy (N-AN, n = 14). RESULTS: The mean age at FMF diagnosis was 7.2 ± 3.0 years. The AN group showed higher rates of hypertension, higher levels of 24-h protein excretion and serum creatinine, and lower estimated glomerular filtration rate at the time of kidney biopsy. The rate of ESRD was found to be higher in the AN group (p = 0.011). Mesangioproliferative glomerulonephritis was the most common pathology in the N-AN group (21.4%). The frequency of amyloidosis was significantly higher in patients with homozygous p.M694V mutations than non-homozygous p.M694V mutations (p = 0.039). CONCLUSIONS: In children with FMF, nephropathy is rare. To our knowledge, this is the first study performed in pediatric FMF patients exploring amyloid and non-amyloid glomerulopathies. Patients with AN had higher rates of proteinuria, lower estimated glomerular filtration rate levels, and higher blood pressure than N-AN patients at the time of biopsy.

A retrospective analysis of children with Henoch-Schonlein purpura and re-evaluation of renal pathologies using Oxford classification.

BACKGROUND: Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aims of this study were to investigate the clinical and laboratory characteristics of our HSP patients, to identify the risk factors for the development of Henoch-Schönlein purpura nephritis (HSPN) and to assess the efficacy of the Oxford Classification system for predicting renal outcomes. METHODS: We performed a retrospective review of HSP patients who admitted to our center between 2001 and 2016, and were < 18 years on admission. RESULTS: A total of 1120 children with HSP were analyzed. Their mean age was 7.4 ± 3.4 years. At onset, purpura was present in all cases, arthritis/arthralgia in 42.4%, abdominal involvement in 39% and renal involvement in 37%. Risk factors for the development of nephritis were age ≥ 8 years, atypical distribution of purpura, ESR > 20 mm/h and abdominal pain. Renal biopsy was performed on 75 patients before immunosuppressive treatment. The mesangial score was strongly associated with proteinuria. Segmental glomerulosclerosis, tubular atrophy/interstitial fibrosis, and crescent formation of ≥ 50% were associated with reduced eGFR at the time of biopsy. A Kaplan-Meier plot showed that segmental glomerulosclerosis and tubular atrophy/interstitial fibrosis significantly predict poor renal outcome. CONCLUSION: The long-term morbidity of HSP is predominantly attributed to renal involvement. Patients with HSP, who have a high risk to develop nephritis, could be followed for longer periods of time. The Oxford classification is useful in predicting long-term outcomes of HSPN.

Symptomatic Fetal-Type Cardiac Rhabdomyoma.

Rhabdomyomas are the most common primary cardiac tumors, especially seen during early periods of childhood. Fetaltype rhabdomyoma is a benign tumor described almost always in extracardiac locations. Although the natural history of the cardiac rhabdomyoma is to regress, the behaviour of the fetal-type rhabdomyomas when present in the heart is unknown with respect to its infrequency. Herein, we report a hemodynamically unstable female neonate with a single large intra-cardiac mass unresponsive to medical treatment, who underwent surgery. The neonate could not survive the operation due to ventricular fibrillation. The mass was diagnosed as fetal-type cardiac rhabdomyoma on autopsy.

Crescentic glomerulonephritis in children: a single centre experience.

BACKGROUND: Crescentic glomerulonephritis (CsGN) is characterized by crescents in 50% or more of glomeruli and clinically by a sudden and progressive decline in renal function. METHODS: We evaluated the etiology, clinical features, prognostic factors and long-term outcome of CsGN. Between January 2000 and December 2010, 45 children (26 girls, 19 boys) with biopsy-proven CsGN (>50% crescents) were investigated retrospectively. RESULTS: The mean age of the patients was 130.86±33.77 months. The mean duration of symptoms prior to diagnosis was 26±12 days (4-40 days). Most of the children had hypertension (62.2%), macroscopic hematuria (73.3%), oligoanuria (44.4%), edema (51.1%) and purpuric rash (40%) at presentation. The final clinical status of the patients was complete remission (n=21), partial remission (n=5) or chronic kidney disease (n=19). Adverse outcomes were significantly associated with a long duration between the onset of symptoms and treatment (P=0.038), the presence of oligoanuria (P=0.006), a severe decreased glomerular filtration rate (GFR <30 mL/min/1.73m²) and the need for dialysis (P=0.003) on admission, the ratio of crescents (>75%) (P=0.03), and the ratio of fibrous crescents (P=0.015). CONCLUSION: The outcome of CsGN in children continues to be poor, and it should be treated as a renal emergency.

Severe leukemoid reaction in a preterm infant with congenital cytomegalovirus infection.

Leukemoid reaction, defined as a total leukocyte count of >50,000/mm, is most commonly related to antenatal administration of steroids, infections, and transient myeloproliferative disorder of Down syndrome in newborns. Atypical presentations of viral infections can be a diagnostic challenge in the newborn period. Cytomegalovirus (CMV) infection causes a multisystem disease, and symptomatic infants generally present with intrauterine growth restriction, hepatosplenomegaly, cholestasis, rash, thrombocytopenia, and microcephaly. We present a case of a preterm infant with severe myeloid leukemoid reaction (leukocyte count >100,000/mm) at birth who was diagnosed with congenital CMV infection on the basis of CMV polymerase chain reaction results after the appearance of cholestasis, blueberry muffin rash, and hepatosplenomegaly.

A rare case of multiple skip segment Hirschsprung's disease in the ileum and colon.

As a rare form of Hirschsprung's disease, skip segment Hirschsprung's disease (SSHD) involves a "skip area" in normally ganglionated intestine, surrounded by aganglionosis. We report a case of multiple SSHD in the ileum and colon with total colonic aganglionosis. To our knowledge, this is the 27th case of SSHD, the third paper on multiple-segment SSHD, and the second patient with SSHD in the ileum to be reported in the English literature.

Solid tumors in Turkish children: a multicenter study.

BACKGROUND: This paper presents a detailed incidence study on childhood solid tumors comprising a histopathology-based documentation of benign and malignant lesions. METHODS: The Ankara Pediatric Pathology Working Group collected databases of pediatric solid tumors from six pediatric reference centers in order to analyze the incidence, distribution and some epidemiologic characteristics of the tumors and to establish a multicenter database for further studies. A five-year retrospective archive search was carried out. Excluding epithelial tumors of the skin, leukemia, lymphoreticular system neoplasias, metastatic tumors, and hamartomas, 1362 solid tumors in 1358 patients were classified according to age, sex, localization, histopathology and clinical behavior. RESULTS: The male/female ratio was 0.9; 14.8% (201) of the patients belonged to 0-1 year age group, 20.7% (281) to 2-4 years, 25.9% (352) to 5-10 years, 22.2% (301) to 11-14 years, and 16.4% (223) to 15-18 years. Among all tumors, 708 (52.0%) were malignant, 645 (47.4%) benign tumors, 2 (0.1%) borderline tumors, and 2 (0.1%) unknown behavioral tumors. Malignant tumors were found in 50.2% (357) of female patients and in 54.0% (349) of male patients. A balanced distribution between benign and malignant entities among children under 18 years was observed. Comparison between the age groups revealed malignant cases outnumbered benign cases under 4 years of age while benign tumor numbers increased after 10 years of age. The most common entities in the malignant group were of sympathetic nervous system origin, while soft tissue tumors far outnumbered the others in the benign group. CONCLUSIONS: We conclude that the cancer patterns of children in the Ankara region mostly resemble with those of the western population. This study provides useful information on the diagnosis of solid tumors in children and highlights variations in cancer incidence in different age groups.

An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old boy, with abdominal distention, pretibial edema, arthritis and petechia on bilateral ankles. He had deteriorated renal functions (creatinine 1.65 mg/dl), hypoalbuminemia (1.6 g/dl) and hypocomplementemia with nephrotic range proteinuria and hematuria. He developed pleural effusion and peritonitis. Serum ANA, anti dsDNA, p ANCA, c ANCA, anticardiolipin IgM and IgG titers were negative. A renal biopsy was performed which revealed diffuse proliferative glomerulonephritis with full-house staining pattern in immunofluorescent microscopic examination suggesting Class IV Lupus Nephritis. He was administered a total of six courses of monthly intravenous pulse methyl prednisolone, dipyridamole, oral cyclophosphamide followed by azothiopirine and oral prednisolone therapy. The renal functions and serum albumin levels turned normal but peritonitis persisted and disappeared after the third pulse steroid therapy. In conclusion, we presented this patient to remind the possibility of SLE in such seronegative patients with unusual findings in order to avoid the delay in the management of this disease with high mortality and morbidity if not treated. Full-house nephropathy is an important clue especially for the diagnosis of ANA negative SLE.

Thyroid nodules in children and adolescents: a single institution's experience.

OBJECTIVE: The aims of this study were to analyze the role of fine-needle aspiration biopsy (FNAB) in the management of pediatric thyroid nodules and to analyze the malignancy risk of thyroid nodules by studying the association between autoimmune thyroiditis and thyroid cancer. METHODS: We conducted a retrospective study on 111 patients with thyroid nodules diagnosed in childhood or adolescence. FNAB was performed in 46 participants with thyroid nodules after ultrasonography (US). Cytology diagnoses were categorized as insufficient, benign, suspicious, and malignant. Clinical and surgical follow-up data were obtained from medical records. The clinical correlation and accuracy of FNABs were evaluated. RESULTS: The family history was positive in four patients. Forty-six patients had positive antithyroid antibodies and an inhomogeneous hypoechogenic US pattern. One patient had previous neck irradiation history. Eighty-six patients (%77.5) were euthyroid. All patients underwent US examination. The FNAB results of the 46 patients were 29 (63%) benign cases, 7 (15%) insufficient, and 10 (22%) suspicious patients. Malignancy was not reported at all. A repetition of FNAB in two benign cases, which were diagnosed with papillary carcinoma during followup, reported these cases as suspicious. Ten patients with suspicious FNAB results underwent surgery because of increases in the size of the nodules; two patients were diagnosed with papillary carcinoma. In this study, the prevalence of malignancy was 4.5% in patients with thyroid nodules. CONCLUSION: In this study, the importance of FNAB in the diagnosis and follow-up of thyroid nodules in childhood has been observed, and risk factors, such as history of familial thyroid carcinoma, radiotherapy to the neck at younger ages, suspicious cytological findings, and increased nodular sizes during follow-up in cases with Hashimoto thyroiditis have been correlated with increased thyroid carcinoma malignancy risk.

Childhood fibroblastic and myofibroblastic tumors: a multicenter documentation and review of the literature.

OBJECTIVE: In this study, we aimed to give a documentation of 37 cases of childhood fibroblastic/myofibroblastic tumors retrieved from the archives of 6 reference centers in Ankara along with a comprehensive review on the subject. MATERIAL AND METHOD: A retrospective archive search was carried out for the period between 2006-2010 in 6 reference centers in Ankara covering patients with ages ranging between 0-18 years. All the tumors categorized under fibroblastic and myofibroblastic group according to World Health Organization criteria were collected. RESULTS: The study comprised 407 soft tissue tumors in total. Fibroblastic/myofibroblastic tumors constituted 9,1 % (37 cases) of these tumors. According to histopathology; 16 cases were categorized as fibromatosis, 8 cases as inflammatory myofibroblastic tumor, 6 cases as infantile fibrous hamartoma, 3 cases as nodular fasciitis and 2 cases as infantile myofibroblastic tumor/myofibromatosis and 1 case as cranial fasciitis. The only malignant case was an infantile fibrosarcoma. CONCLUSION: Infantile fibrosarcoma was lower than reported series and a male predominance was noted. The low incidence of newly described entities as well suggests that these tumors may have been unrecognized.

Development of a Hodgkin disease tumor in the neck of a patient who previously had undergone complete excision of a hyaline-vascular Castleman disease neck mass.

Castleman disease is an uncommon cause of a neck mass. A benign lymphoproliferative disorder, it may be seen as a self-limited unicentric process or as a fulminant multicentric disease with systemic symptoms. The association between Hodgkin disease and Castleman disease has been debated extensively, but this association is rare. The associated Hodgkin disease frequently has been of the interfollicular subtype and typically has coexisted with the multicentric plasma-cell variant of Castleman disease. We report a case of mixed-cellularity Hodgkin disease of the neck in a patient previously diagnosed with hyaline-vascular-type Castleman disease who had undergone complete excision of a neck mass 2 years earlier.

Vascular endothelial growth factor, vascular endothelial growth factor receptor-3 and cyclooxygenase-2 expression in psoriasis.

OBJECTIVE: To investigate expression patterns and relationship of vascular endothelial growth factor (VEGF), vascular endothelial receptor-3 (VEGF-R3) (FLT-4) and cyclooxygenase-2 (COX-2) in psoriasis. STUDY DESIGN: Forty-three patients were included in this study. The clinical severity of psoriasis was assessed using the psoriasis area and severity index (PASI). Punch biopsy samples both from psoriatic and nonlesional skin were taken and VEGF, VEGF-R3 and COX-2 expressions determined. RESULTS: VEGF, VEGF-R3 and COX-2 expressions were detected in 90.9%, 78.0% and 86.4% of psoriatic and 84.1%, 71.8%, and 84.1% of nonlesional skin, respectively. Epidermal VEGF, VEGF-R3 and COX-2 expressions were detected in 56.8%, 77.8% and 34.1 of psoriatic and 75%, 78.1% and 65.9% of nonlesional skin, respectively. In dermis, VEGF, VEGF-R3 and COX-2 expression was observed in 88.6%, 77.5% and 84.1% of psoriatic and 81.8%, 64.1% and 77.3% of nonlesional skin, respectively. Among the PASI subgroups no statistically significant differences were detected for VEGF, VEGF-R3 and COX-2 expression. CONCLUSION: Our study demonstrated that VEGF, VEGF-R3 and COX-2 expression in psoriatic and nonlesional skin is significantly high in epidermis and dermis. Although there was significant concordance between VEGF and VEGF-R3 expressions in psoriatic lesions, there seems to be no concordance between the others.

Expressions of vascular endothelial growth factor and CD34 in oral aphthous lesions of Behçet's disease.

OBJECTIVE: Behçet's disease (BD) is an immunoin-flammatory vasculitis with an unknown etiopathogenesis. Vascular endothelial growth factor (VEGF) is a cytokine-stimulating angiogenesis. It has been suggested to play a role in inflammation and pathogenesis of vasculitic processes. STUDY DESIGN: VEGF and CD34 expressions were assessed in samples taken from oral aphthous lesions. The patients were evaluated for disease activity, duration of lesions, serum C-reactive protein (CRP) levels and erythrocyte sedimentation rate (ESR). RESULTS: Twenty-six patients were included. Fourteen (53.9%) had increased CRP levels and 12 (46.1%) had increased ESR levels. Positive VEGF and CD34 staining were detected in 46.2% and 69.2% of biopsy samples, respectively. There was good positive correlation between them. The frequency of positive VEGF and CD34 staining was statistically higher in lesions with a duration of more than 6 days. No correlation was found between positive VEGF staining and serum CRP level, ESR and disease activity. CONCLUSION: Increased VEGF expression in correlation with CD34 positivity in oral aphthous lesions may show the role of VEGF in pathogenesis of these vasculitic lesions. We have concluded that VEGF may play a role during the course of oral aphthous lesions in BD.

Brucella glomerulonephritis: case report and review of the literature.

The authors present the case of a 17-year-old shepherd who was diagnosed with diffuse proliferative glomerulonephritis and diffuse tubulointerstitial nephritis during the course of Brucella infection. The pathogenesis and the mechanism of renal involvement in brucellosis is discussed in light of the pertinent literature.

An unusual site for an intraoral schwannoma: A case report.

Schwannomas are benign tumors that are rarely found in the oral cavity. We presented a schwannoma arising from the parasympathetic fibers of the lingual nerve, which is the first in the literature to our knowledge. Its borders in computerized tomography are not well defined despite the contrast medium.

Giant cell reparative granuloma originating from the ethmoid sinus.

Giant cell granuloma is a reactive osseous proliferation and is histologically benign despite its aggressive clinical course. It usually involves the maxilla and the mandible in the region of the head and neck. It is rare in the ethmoid region. In this paper, an aggressive and recurrent giant cell reparative granuloma, which is recurred in a very short time period, originating from the ethmoid sinus is presented and the criteria for the differential diagnosis are noted in the light of the histological, clinical and biochemical parameters and the literature is reviewed.