1,25-dihydroxyvitamin D-mediated hypercalcemia in ovarian dysgerminoma

Humoral hypercalcemia of malignancy (HHM) is a rare complication of malignant pediatric tumors, specifically those that secrete humoral factor(s), such as parathyroid hormone-related peptide (PTHrP). The authors report a case of severe hypercalcemia associated with ovarian dysgerminoma in a 10-year-old girl. In this case, the humoral factor was considered to be 1,25-dihydroxyvitamin D. HHM is extremely resistant to medical therapy. Therefore, tumor resection or volume reduction is necessary to control serum calcium levels.     

1,25-DIHYDROXYVITAMIN D-MEDIATED HYPERCALCEMIA IN OVARIAN DYSGERMINOMA

Humoral hypercalcemia of malignancy (HHM) is a rare complication of malignant pediatric tumors, specifically those that secrete humoral factor(s), such as parathyroid hormone-related peptide (PTHrP). The authors report a case of severe hypercalcemia associated with ovarian dysgerminoma in a 10-year-old girl. In this case, the humoral factor was considered to be 1,25-dihydroxyvitamin D. HHM is extremely resistant to medical therapy. Therefore, tumor resection or volume reduction is necessary to control serum calcium levels.     

Hypercalcemia and carcinoma of the penis

Hypercalcemia is the most common metabolic disorder associated with malignancies. Squamous cell carcinoma of the penis is a tumor for which this abnormality has rarely been described. This report presents a case of hypercalcemia seen in a patient with advanced penile cancer. A chemotherapy regimen of intravenous cisplatin and fluorouracil caused regression of the primary tumor and normalization of the serum calcium. A literature review supported an association between squamous cell carcinoma of the penis and hypercalcemia. Med. Pediatr. Oncol. 29:576–577, 1997. © 1997 Wiley-Liss, Inc.     

Severe malignancy-associated hypercalcemia in dysgerminoma

Hypercalcemia is a rare complication of pediatric malignant germ cell tumors. The problem may be missed because of unawareness among pediatric oncologists. We describe a 16-year-old girl with an ovarian dysgerminoma associated with severe hypercalcemia, a metabolic abnormality infrequently reported with this disease. We review some of the potential causes of malignancy-associated hypercalcemia and current treatment strategies. It is our recommendation that calcium levels should be monitored in all children with solid ovarian masses. Hypercalcemia seen in these situations may not improve until the tumor is removed.     

Concomitant hypercalcemia and hyperammonemia associated with distal renal tubular acidosis

We describe an infant with concomitant hypercalcemia and hyperammonemia associated with nonanion gap metabolic acidosis secondary to distal renal tubular acidosis (dRTA). The levels of both serum calcium and ammonia rapidly normalized with the correction of dehydration and metabolic acidosis. To the best of our knowledge, there has been only one previous case report of concomitant hypercalcemia and hyperammonemia associated with dRTA that has been reported in the literature. We describe the causes and emergent management of hypercalcemia and review the possible mechanisms of this rare association with dRTA.     

Dysgerminoma in Mother and Daughter: Use of Lactate Dehydrogenase as a Tumor Marker in the Child

A 7-year-old girl presented with an extragonadal dysgerminoma arising from the pelvis. Her mother had been treated for a histologically identical pituitary tumor 3 years previously. The child's serum lactate dehydrogenase (LDH) level was markedly elevated at presentation and fell as the tumor responded to treatment. The potential use of LDH as a marker for gonadal dysgerminoma is well documented, but raised LDH in association with primary extragonadal dysgerminoma has not been described previously. In addition, this is the first report of extragonadal dysgerminoma occurring in female relatives.     

Altered mental status and hematemesis in a child with hypercalcemia

Altered mental status in a child is a potentially life-threatening condition with a broad differential including vascular, toxin-mediated, infectious, metabolic, and traumatic causes. Hypercalcemia is a rare cause of altered mental status in children. We report a case of a 13-month-old boy with familial hypocalciuric hypercalcemia who presented to our emergency department with altered mental status and hematemesis. Familial hypocalciuric hypercalcemia is a rare cause of hypercalcemia that usually presents with asymptomatic hypercalcemia. This case illustrates the presentation of severe hypercalcemia and reviews the initial management and evaluation of hypercalcemia in children.     

Hypercalcemia revealing iatrogenic hypervitaminosis A in a child with autistic troubles]

Hypervitaminosis A is an unusual cause of infant hypercalcemia. The way it occurs can be very surprising, as one can notice from the following case report. CASE REPORTS: A three-year-old boy, presenting important behavioral disorders, was hospitalized because of a deterioration of his general state of health associated with vomiting, cephalgias, fever and cutaneous abnormalities. A 168 mg/L hypercalcemia was found. The only etiology is a deviant consumption of vitamin A within the framework of an "autistic diet": 100000 UI/d during three months, and then 150000 UI/d the three following months. Intoxication was confirmed by the increased vitamin A plasmatic level, and vitamin A/RBP molar ratio and by the presence of plasmatic retinyl palmitate. An emergency treatment by rehydration, biphosphonates and furosemide led to effective calcemia normalization. CONCLUSION: In the case of nonobvious causes of hypercalcemia, a thorough cross-examination must look for vitamin A intoxication. Our observation illustrates the danger of certain diets suggested for autistic children.     

Dysgerminoma in Mother and Daughter: Use of Lactate Dehydrogenase as a Tumor Marker in the Child

A 7-year-old girl presented with an extragonadal dysgerminoma arising from the pelvis. Her mother had been treated for a histologically identical pituitary tumor 3 years previously. The child's serum lactate dehydrogenase (LDH) level was markedly elevated at presentation and fell as the tumor responded to treatment. The potential use of LDH as a marker for gonadal dysgerminoma is well documented, but raised LDH in association with primary extragonadal dysgerminoma has not been described previously. In addition, this is the first report of extragonadal dysgerminoma occurring in female relatives.     

Hypercalcemia in association with mesoblastic nephroma: report of a case and review of the literature

Hypercalcemia, often associated with certain types of adult tumors, has also been described in pediatric neoplasms. In childhood, the more common associations include lymphoma, leukemia, rhabdomyosarcoma and rarely neuroblastoma. However, recently, several infants with hypercalcemia were described having renal tumors without bone metastases. The following is a case report of a 2-month-old infant who presented with severe hypercalcemia and a large right-sided abdominal mass, which at surgery was diagnosed as a cellular mesoblastic nephroma.     

Severe hypercalcemia associated with Williams syndrome successfully treated with pamidronate infusion therapy

Infantile hypercalcemia becomes manifest in 15% of patients with Williams syndrome (WS) and generally is not clinically severe. However, some patients with WS can have severe hypercalcemia and do not respond well to traditional therapies. Recently, pamidronate has been used in the treatment of childhood hypercalcemia associated with many disorders, but there is little experience with the treatment of hypercalcemia with bisphosphonates in patients with WS. We present a 17-month-old female patient, who had been diagnosed as WS by genetic analysis, admitted to our clinic for the investigation of severe hypercalcemia (4.02 mmol/L). Because the patient did not respond very well to fluid administration, furosemide infusion, and dietary calcium restriction, pamidronate infusion was performed and calcium levels returned to normal within 2 days. This case report is presented to point out that pamidronate therapy seems to be a safe and efficient way of treating life-threatening hypercalcemia in WS.     

Recurrence of a dysgerminoma in Frasier syndrome

Mestrallet G, Bertholet‐Thomas A, Ranchin B, Bouvier R, Frappaz D, Cochat P. Recurrence of a dysgerminoma in Frasier syndrome.
Pediatr Transplantation 2011: 15:e53–e55. © 2010 John Wiley & Sons A/S. Abstract: FS is an inherited disease characterized by male pseudohermaphroditism and glomerular involvement leading to end‐stage renal disease during adolescence or early adulthood (J Pediatr 1964:64:740). The FS phenotype in 46,XY patients consists of female external genitalia, gonadal dysgenesis, high risk of gonadoblastoma, and development of renal failure in the second decade of life. FS is caused by heterozygous mutation in intron 9 of the WT1 leading to a change in splicing that results in loss of three amino acids (+KTS isoform), thus disrupting the normal ratio of the +KTS/?KTS isoforms that is critical for proper gonadal and renal development (Nat Genet 1997:17:467; Hum Mol Genet 1998:7:709). We report on a patient followed for FS revealed by acute peritoneal syndrome because of ovarian dysgerminoma. Therapeutic options had led to an unusual course with recurrent neoplastic disease after renal transplantation.     

Neonatal subcutaneous fat necrosis with hypercalcemia: efficiency of a low dose of corticosteroids]

We report the case of a newborn with macrosomia, extensive subcutaneous fat necrosis and symptomatic hypercalcemia. Low doses of prednisone were efficient, while dietary intervention, hyperhydratation and furosemide were not. Treatment of hypercalcemia in this specific neonatal condition are discussed.     

Germinoma of the pineal body with syncytiotrophoblastic giant cells

Almost all types of germ cell tumors are known to occur in the third ventricle-pineal region. In this paper we report the case of a 16-year-old boy having a particular subtype of germinoma of the pineal body in which scattered syncytiotrophoblastic cells positive for human chorionic gonadotropin were found. This tumor is considered the central nervous system counterpart of seminoma with syncytiotrophoblastic giant cells and dysgerminoma with syncytiotrophoblastic giant cells.     

Etidronate therapy for hypercalcemia in subcutaneous fat necrosis of the newborn

Subcutaneous fat necrosis of the newborn (SCFN) is characterized by indurated violet skin nodules and, occasionally, life-threatening hypercalcemia. Current treatments of patients with SCFN-related hypercalcemia are often only partially successful and may be associated with prolonged hypercalcemia. We now report the use of etidronate, a bisphosphonate, to control hypercalcemia in an infant with SCFN.     

Hypercalcemia in acute lymphatic leukemia

We report the case histories of 2 patients with a rapidly fatal form of acute lymphatic leukemia, who showed severe hypercalcemia during the acute phase of the disease. The pathogenetic mechanism of the hypercalcemia in these two cases was different. A short review of present day theories regarding the pathogenesis of tumor hypercalcemia is presented.     

Pamidronate as first-line treatment of hypercalcemia in neonatal subcutaneous fat necrosis: A case series

BackgroundSubcutaneous fat necrosis (SCFN) can be complicated by severe hypercalcemia, which is frequently asymptomatic. Nephrocalcinosis is associated with hypercalcemia and, in other clinical settings, has been linked to furosemide and glucocorticoid use. First-line bisphosphonate therapy treating hypercalcemia in neonatal SCFN is not well described.ObjectivesTo describe the biochemical changes and risk of nephrocalcinosis in infants with hypercalcemia, secondary to neonatal SCFN, treated with initial pamidronate.MethodsA retrospective chart review of five infants treated with initial pamidronate and without furosemide or glucocorticoids. Data were collected on the following: timing of presentation, therapeutic response, and presence of nephrocalcinosis.ResultsHypercalcemia resolved after 2.8±1.7 days; this is compared to 7.6±2.8 days from previously reported cases utilising alternative therapies (P=0.012). There were no episodes of rebound hypercalcemia or hypocalcemia. Nephrocalcinosis was present in four of five cases. When including published cases, age at diagnosis was associated with presenting serum calcium (P=0.003) and nephrocalcinosis was associated with higher serum calcium (P=0.014) and time from SCFN to hypercalcemia diagnosis (P=0.002).ConclusionsThis retrospective case series demonstrates that first-line pamidronate treatment was effective and safe in the resolution of hypercalcemia. Nephrocalcinosis was observed, despite the avoidance of furosemide and glucocorticoid therapy, and associated with greater disease severity and duration of hypercalcemia.     

Dysgerminoma in a child with ataxia-telangiectasia

Ataxia-telangiectasia is an autosomal recessive disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, high incidence of cancer, and increased sensitivity to ionizing radiation. The authors report a case of dysgerminoma in a child with high alpha-fetoprotein, CA125 and beta-human chorionic gonadotropin, who has been followed-up for ataxia-telangiectasia for 2 years.     

Asymmetric medullary nephrocalcinosis in two children

Medullary nephrocalcinosis (MNC) is usually a bilateral process with symmetric involvement of both kidneys. Asymmetric medullary nephrocalcinosis has been previously reported in the literature, but has not been well illustrated or explained. We report the sonographic findings in two pediatric patients with hypercalcemia. In both patients an unrelated unilateral renal abnormality, (renal vein thrombosis in one and obstructive hydronephrosis in the other) prevented the development of MNC in the affected kidney, probably by decreasing the glomerular filtration rate and/or altering the renal tubular function. Received: 23 December 1997 Accepted: 16 March 1998