Chemotherapy-associated Raynaud's phenomenon in a two-year-old girl

Raynaud's phenomenon occurred in a 2-year-old girl 17 months after starting multiagent chemotherapy for an endodermal sinus tumor of the vagina. Symptoms began 2 months after receiving her final dose of bleomycin, and gradually resolved over a 6-month period despite continuing vinblastine. Chemotherapy-associated Raynaud's phenomenon appears to be unusual in young children, although mild episodes of vasospasm may not be reported by children or their parents. Specific questions regarding symptoms of Raynaud's phenomenon should be asked of all children receiving vinblastine and bleomycin therapy.     

Teratoma in an adolescent with malignant transformation into embryonal rhabdomyosarcoma: case report

BACKGROUND: The somatic type tumors are occasionally found in nonseminomatous germ cell tumors in men. These malignancies are presumed to arise from malignant transformation (MT) of teratoma or by differentiation of totipotential germ cell. OBSERVATION: A case of MT of germ cell tumor in 17-year-old male into embryonal rhabdomyosarcoma is described. The histopathologic diagnosis was that of embryonal rhabdomyosarcoma in which no germ cell elements were found. The germ cell origin of transformed histology is supported by cytogenetic analysis (isochromosome 12p), and elevated alpha(1)-fetoprotein. Despite intensive therapy the patient died. CONCLUSIONS: MT of teratoma is rare entity with poor prognosis.     

Delayed diagnosis in an adolescent with a malignant testicular tumour

Feelings of embarrassment and fear in adolescents may contribute substantially to a delay in the diagnosis of malignant tumours arising from the genital region, with subsequent increase in morbidity and mortality. A case of a 15-year-old boy who had multiple visits to physicians with complaints of recurrent lower abdominal and back pain is presented in the current case report. He refused examination of his genital area during all visits, stating that there were no problems. The patient was admitted to the hospital with a working diagnosis of lymphoma after finding an abdominal mass and a neck mass. During bone marrow aspiration, while the patient was sedated, a large testicular mass was discovered. Each time the patient had been examined, he refused a genital examination and denied any change in the genital region.     

Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine

PURPOSE: Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of vincristine in the treatment of KMP. METHODS: We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe. RESULTS: All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability. CONCLUSION: Vincristine presents a safe and sometimes effective treatment option in the management of KMP.     

Implantation metastasis of primary malignant rhabdoid tumor of the brain in an adult (one case report)

Primary malignant rhabdoid tumor (PMRT) of the brain is a rare and recently described neoplasm of youth. We report magnetic resonance imaging (MRI), computed tomography (CT), and pathology of one case of PMRT in an adult which seeded along the needle track for stereotactic biopsy. Med. Pediatr. Oncol. 28:223–227 © 1997 Wiley-Liss, Inc.     

Virilizing ovarian tumor in an adolescent

This clinical case describes a 13 year-old pubertal girl suffering from secondary virilization, amenorrhea, and abdominal mass. Plasma testosterone and androstenedione levels were markedly elevated. Upon surgery, an ovarian tumor containing 5 l of liquid was removed. The histologic examination revealed a macrocystic granulosa cell type tumor of non-juvenile type. After removal of the tumor, which included ipsilateral ovariectomy, the contralateral ovary developed polycystic changes. The role of the tumoral hyperandrogeny in inducing this contralateral polycystic ovary is discussed.     

Anti-centromere antibodies as a marker of Raynaud's phenomenon in pediatric rheumatologic diseases

To examine the possible relationship between anti-centromere antibodies (ACA) and pediatric rheumatologic diseases, we investigated the presence of ACA (using enzyme immunoassay) in the sera of 45 children and adolescents with such diseases and compared the results with a group of 42 age- and gender-matched healthy subjects. ACA were present (≥ 10 U/ml) in three out of five patients (60%) with scleroderma (SCD), in seven out of 16 (43.8%) patients with systemic lupus erythematosus (SLE), in two out of five patients (40%) with mixed connective tissue disease (MCTD), in one out of four patients (25%) with dermatomyositis (DMS), and in two out of 14 patients (14.3%) with juvenile rheumatoid arthritis (JRA). ACA were also detected in a single patient with anti-phospholipid syndrome (APL) who had digital gangrene and hemiparesis, as well as in two healthy subjects. ACA positivity was related to the presence of Raynaud's phenomenon in the studied sample, as 86% of patients suffering from the phenomenon were ACA positive. ACA positivity was associated with older age, high blood pressure and high erythrocyte sedimentation rate (ESR) values, and lower hemoglobin and weight and height percentile values. It was also higher among anti-nuclear antibody-positive subjects. Raynaud's phenomenon and ACA positivity shared almost the same clinical and laboratory associations in the studied patients. Thus, ACA are probably among the markers of Raynaud's phenomenon in pediatric rheumatologic diseases. Their value as predictors of future development of the phenomenon needs further evaluation.     

Hemangiopericytoma in an adolescent girl: a case report

Hemangiopericytoma (HPC) is a rare soft tissue tumor. The few published reports account for the little information available on its clinical management. Here the authors report the successful treatment of an adolescent girl with rare HPC of the tongue. After incomplete surgical excision of the tumor she was admitted to the Hematology-Oncology Department and was treated with a 3-drug combination regimen (ifosfamide, actinomycin D, vincristine) for 8 weeks. She achieved partial remission in week 9 based on the magnetic resonance imaging (MRI) findings. Conventional radiation therapy was initiated at week 9 and continued until week 16. At week 20, according to the MRI findings, she achieved complete remission and continuation therapy was initiated. The young girl has been alive without evidence of the disease for the last 3 years of follow-up. In conclusion, the current report indicates that in cases of incomplete surgical excision of the tumor, chemotherapy and radiotherapy seem to be effective.     

Benign breast lesions in adolescent girls: an overview with a case report

A wide spectrum of breast disorders occurs in the paediatric and adolescent age groups, but malignant disease is very rare. The relative frequencies of these conditions and their natural history differ substantially from those of adult patients. The gross findings may be very deceptive and mislead the clinician until a histopathological diagnosis is made. We report a case in an adolescent girl that was initially diagnosed and treated as fibroadenoma until a histological assessment proved it to be a phyllodes tumour with malignant potential.     

Raynaud's phenomenon of the nipple: a treatable cause of painful breastfeeding

Maurice Raynaud first described the vasospasm of arterioles in 1862, and Raynaud's phenomenon is now felt to be common, affecting up to 20% of women of childbearing age. Raynaud's phenomenon has been reported to affect the nipples of breastfeeding mothers and is recognized by many lactation experts as a treatable cause of painful breastfeeding. In 1997, Lawlor-Smith and Lawlor-Smith reported 5 women with Raynaud's phenomenon associated with breastfeeding, but there are few other case reports, and none report the possible relationship between Raynaud's phenomenon of the nipple and previous breast surgery. We report 12 women who breastfed 14 infants, all of whom were seen in 1 pediatric practice and 1 lactation consultation center in San Francisco, California, within the past 3 years. Of the 12 women, 11 were seen between June 2002 and May 2003. All women suffered from extremely painful breastfeeding, with symptoms precipitated by cold temperatures and associated with blanching of the nipple followed by cyanosis and/or erythema. Poor positioning and poor attachment or latch may cause blanching of the nipple and pain during breastfeeding, but 10 of the 12 mothers were evaluated by experienced lactation consultations, who were sure that inappropriate breastfeeding techniques were not contributing factors. Because the breast pain associated with Raynaud's phenomenon is so severe and throbbing, it is often mistaken for Candida albicans infection. It is not unusual for mothers who have Raynaud's phenomenon of the nipple to be treated inappropriately and often repeatedly for C albicans infections with topical or systemic antifungal agents. Eight of our 12 mothers and their infants received multiple courses of antifungal therapy without relief before the diagnosis was made. To diagnose Raynaud's phenomenon accurately, additional symptoms such as precipitation by cold stimulus, occurrence of symptoms during pregnancy or when not breastfeeding, and biphasic or triphasic color changes must be present. All our mothers experienced precipitation of symptoms by cold stimuli and demonstrated biphasic or triphasic color changes, and 6 of the 12 experienced symptoms during pregnancy. Interestingly 3 of 12 mothers also reported a history of breast surgery, including 1 mother who had a fibroadenoma removed and 2 who had breast-reduction surgery. The association between breast surgery/implants and autoimmune disease, including Raynaud's phenomenon, has been discussed extensively, but the association of Raynaud's phenomenon of the nipple during breastfeeding has not been reported previously. Given the small numbers in the study, it is uncertain as to whether this may be a precipitating factor in developing Raynaud's phenomenon. Treatment options include methods to prevent or decrease cold exposure, avoidance of vasoconstrictive drugs/nicotine that could precipitate symptoms, and pharmacologic measures. There are reports in the lay press of the use of herbal medicines, aerobic exercise, and dietary supplements, but because most women with painful breastfeeding require immediate relief of the pain to continue breastfeeding successfully, it is important to offer a treatment plan that will alleviate the pain quickly. Nifedipine, a calcium channel blocker, has been used to treat Raynaud's phenomenon because of its vasodilatory effects. Very little of the medication can be demonstrated in breast milk and thus is safe to use in breastfeeding mothers. Of the 12 mothers in our series, 6 chose to use nifedipine, and all had prompt relief of pain. Only 1 mother developed side effects from nifedipine. Pediatricians and lactation consultants should be aware of this treatable cause of painful breastfeeding and should specifically question their patients, because most mothers will not provide this information to the breastfeeding consultant. Prompt treatment will allow mothers to continue to breastfeed pain free while avoiding unnecessary antifungal therapy.     

Addisonian crisis in an adolescent female: a case report

Acute adrenal insufficiency is rarely diagnosed in the pediatric age group. In this case report, an 11-year-old female with three previous admissions for dehydration was seen for dehydration and shock. Adrenal insufficiency was considered as a working diagnosis in the emergency department. Rapid intervention with intravenous hydrocortisone and fluid resuscitation resulted in a good outcome.     

Mycoplasma pneumoniae infection. Manifestation in a 3-year-old child by Raynaud's phenomenon

The authors report the case of a 3 year-old boy with mycoplasma pneumoniae infection. The presenting sign was Raynaud's phenomenon with cryoglobulinemia and cryofibrinogenemia. To their knowledge this is the first reported case presenting with such an association.     

Multiple aggressive vertebral haemangiomas in an adolescent: a case report

We describe a neurologically symptom-free adolescent with aggressive vertebral body haemangiomas at two sites. Received: 1 August 1997 Accepted: 14 August 1997