Subcutaneous (deep) granuloma annulare in children: a possible mimicker of epithelioid sarcoma

Subcutaneous granuloma annulare (SGA) is a self-limited inflammatory lesion consisting of dermal or subcutaneous nodules usually affecting children. Lower extremity involvement is the most common anatomic site. Because of the subcutaneous location, the morphological diagnosis of SGA can be challenging, and differential diagnoses are both benign and malignant processes including epithelioid sarcoma. Our article examines the clinical, histopathological, and immunohistochemical aspects of SGA in comparison to ES. We present 3 cases of SGA in children, who were initially diagnosed with ES and discuss the differential diagnoses features between SGA and ES. Because SGA can simulate ES, the awareness of this possibility is important to avoid overtreatment, like amputation, of the benign condition (SGA).     

Candida Funisitis: A Clinicopathologic Study of 32 Cases

We report on 32 cases of Candida funisitis and describe the associated clinicopathologic features. The Candida funisitis was characterized grossly by small, circumscribed, yellow-white nodules on the umbilical cord surface and, microscopically, by subamnionic microabscesses in which fungal organisms were demonstrable. Chorioamnionitis was present in all cases. Twenty-four (75%) of the 32 infants were premature. There were 7 perinatal deaths, all in immature fetuses. Five (16%) of the 32 fetuses had congenital candidiasis. Five (16%) of the mothers had a history of intrauterine foreign body, including intrauterine contraceptive device in three and cervical cerclage in two. The diagnosis of Candida funisitis should prompt a careful examination for fetal infection, even though it is associated with congenital candidiasis in only a minority of the cases. Received January 17, 1997; accepted April 2, 1997     

Histopathological Features of Acral Melanocytic Nevi in Children: Study of 21 Cases

Benign melanocytic lesions in children may give cause for some concern histologically. This is because they represent a specific entity, or they reflect the state of evolution of the lesion or the anatomical location. This latter phenomenon has been poorly documented in children. In this study, we address the problem of atypical features frequently seen in benign nevi from acral sites in a group of patients aged 18 years or less. Twenty-one cases (12 female, 9 male) were identified from the Department of Pathology files during the years 1975–1988. All were Caucasian. Histological examination revealed that 6 cases were congenital and 15 were aquired; of these, 19 cases (90%) had a junctional component and all of these exhibited architecture atypia in the form of either lentiginous proliferation (84%) or confluence of junctional nests (84%). Forty-two percent (8/19) showed a mixture of both. Thirty-seven percent (7/19) exhibited transepidermal elimination of melanocytic nests, with 13/19 (68%) showing single cell infiltration of the epidermis. Atypical size, shape, and location of the junctional nests were present in 10/19 cases (53%). Within this group there appears to be no relationship between the age of the patient and the degree of architectural atypia. Mild cytological atypia was common. This report stresses the importance of anatomic subsite in the assessment of melanocytic lesions in children as well as in adults. Received July 18, 1997; accepted December 1, 1997.     

Peripheral Neurogenic Tumors of the Soft Tissues in Children and Adolescents: a Clinicopathologic Study of 139 Cases

A review of over 900 soft tissue neoplasms in children and adolescents revealed 139 neurogenic tumors in 122 patients from newborn to 20 years of age, which had been accessioned in a 2 5-year period. Based upon clinicopathologic criteria, 84 (60%) tumors were regarded as unequivocally benign or borderline and the remaining 55 (40 %) were malignant. The average age at diagnosis for the entire series was 10 years; the sex distribution was approximately equal; and the trunk (66, 48%) was the most frequent topographic site, followed by the head and neck (39, 29%) and extremities (31, 23%). Eighteen tumors (13%) were recognized at birth. Twenty-one patients (17%) had the stigmata of von Recklinghausen's neurofibromatosis (VRN). Neurofibromas and cellular peripheral nerve sheath tumors accounted for 43% (60 cases) of the diagnoses; these 60 neoplasms occurred in 41 patients, 12 of whom had VRN. The second largest category of neoplasms (38 cases, 28%) was the primitive neuroectodermal tumor (PNET) including the subset of “malignant small cell tumor of thoracopulmonary origin” or Askin tumor. The PNETs other than the Askin tumor (26 cases) presented mainly on the trunk (63%) and head and neck region (33 %), showed a male predilection (58 %) and had a mean age at diagnosis of 7 years. Askin tumors (12 cases) were, by definition, confined to the chest wall or thoracic cavity, showed a female predilection (71%), and presented in the second decade (82%, mean age 14 years). There were 16 (11%) malignant peripheral nerve sheath tumors, seven of which occurred in children with VRN. Six other categories of neurogenic tumors constituted the remaining cases. Our findings indicate that neurogenic neoplasms are an important nosologic group of soft tissue tumors in the pediatric population.     

小儿急性出血坏死性胰腺炎的治疗

６例小儿急性出血坏死性胰腺炎，经手术治疗，治愈５例，死亡１例。对其降低死亡率、严格控制禁食时间、营养支持、腹腔引流及灌洗、“三造瘘”等问题加以讨论。     

Mesothelial and Related Neoplasms in Children and Adolescents: A Clinicopathologic and Immunohistochemical Analysis of Eight Cases

Mesothelioma is a neoplasm that occurs infrequently in childhood: only an estimated 2-5% of all cases present in the first two decades of life. The diagnosis may be perplexing because of its rarity and its pathologic similarities to other papillary or spindle cell neoplasms in the pediatric age group. We have studied eight cases of mesothelial or submesothehal-derived neoplasms of pleural (four cases) and peritoneal (four cases) origin in patients 4 to 17 years of age at diagnosis. Microscopically, six were epithelial, with papillary, tubuloglandular, and solid patterns. Two tumors were predominantly fibrous appearing, one a localized pleural fibroma and the other a diffuse pleural sarcomatoid mesothelioma. All of the tumors were immunoreactive for vimentin, and all except the pleural fibroma stained for either cytokeratin, epithelial membrane antigen, or both. None reacted with antibodies to carcinoembryonic antigen, placental alkaline phosphatase, or Leu-M1. At last follow-up, three patients were dead of tumor, three were alive and well, and two had been recently diagnosed and were undergoing treatment. These results indicate that the immunohistochemical profile delineated for mesothelioma in adults is equally applicable to mesothelial neoplasms in younger patients and is useful in establishing a diagnosis. The prognosis for malignant mesothelioma in childhood appears to be as unfavorable as the adult counterpart, with the possible exceptions of certain clinicopathologic subtypes.     

Cutaneous Lymphoblastic Lymphoma in Children: Report of Six Cases with Precursor B-cell Lineage

Precursor B lymphoblastic lymphomas (B-LBL) are generally rare, but appear to have a higher incidence in children than in adults. In this report, we describe in detail six cases of B-LBL presenting with cutaneous lesions. Three occurred in the scalp, one in the skin of the thigh, one in the skin of the face and breast, and one in the subcutaneous tissue of the orbit. All six patients are females ranging in age at presentation from 5 to 15 years (mean = 9.6). None of the cases had bone marrow involvement, while two had bone involvement (maxilla, distal tibia, and distal humerus in one case, and distal tibia and orbital bone in another case); only one case had lymphadenopathy (retroperitoneal). Immunohistochemical staining showed positivity for CD79a and CD43 in all six cases. LCA and L26 positivity were also each seen in one case. Staining for MIC-2 (CD99) showed strong positivity in three cases. Vimentin was positive in four cases and TdT was positive in all five patients tested. Staining for keratin, UCHL-1, or CD30 was not encountered. Cases in which cell marker studies by flow cytometry were performed showed positivity for CD10, CD19 with negative CD20, pan-T-cell, and myeloid markers. The five patients who received multiagent chemotherapy are alive with follow-up intervals of 2 to 18 years. Two patients had local recurrences and were given radiation therapy (one with repeating multiagent chemotherapy). One patient (diagnosed in 1962) died of disseminated disease; she had been treated with radiation therapy and 6MP only. Cutaneous B-LBL must be included in the differential diagnosis of small blue cell tumors, especially in children. In contrast to its T-cell counterpart, B-LBL occurs more frequently in females, tends to present as skin or bone lesions, and is associated with a potential cure, even in cases that relapse. Received June 25, 2001; accepted September 4, 2001.     

Fibroblastic-Myofibroblastic Tumors in Children and Adolescents: A Clinicopathologic Study of 108 Examples in 103 Patients

A review of over 900 soft tissue tumors in children and adolescents revealed 108 fibroblastic-myofibroblastic tumors in 103 patients from newborn to 20 years of age, which had been accessioned in a 25-year period. Based on clinicopathologic criteria, 82 (76%) were regarded as benign, 14 (13%) as borderline, and 12 (11%) as malignant. The average age at diagnosis for the entire series was 7 years with a male/female ratio of 1.8:1. The most frequent topographic site was the extremities (48, 44%), followed by the trunk (31, 29%) and the head and neck region (27, 25%). Virtually 50% (51 tumors) of cases were diagnosed during the first year of life, and 73 (71%) occurred in the first decade. The known recurrence rate was 16% (17 cases). Fibromatoses of various subtypes accounted for 95% of the histologically benign group. Infantile myofibromatosis was the most frequent form of fibromatosis, followed by aggressive (desmoid) fibromatosis (20 cases, 19%). Ninety percent of infantile myofibromatoses were diagnosed in the first year of life. In contrast, 70% of aggressive fibromatoses occurred in the second decade. Associated conditions included familial desmoid fibromatosis, Gardner syndrome, and previous surgery. The borderline category was represented by the 14 (13% of the series) congenital-infantile fibrosarcomas. All of the 14 (13%) malignant tumors were classic adult-type fibrosarcomas that occurred only in later childhood and adolescence. Fibromatosis colli, fibrous hamartoma of infancy, juvenile nasopharyngeal angiofibroma, Dupuytren-type fibromatosis, infantile digital fibromatosis, juvenile aponeurotic fibroma, unclassified fibromatoses, and fibroma of tendon or nerve sheath constituted the remaining cases.     

儿童骨盆骨折的合并伤

回顾了３５例骨盆骨折患儿的临床资料，对其合并伤的原因、特点、诊断和治疗进行了讨论分析。     

Pathology of Suspected Acquired Immune Deficiency Syndrome in Children: A Study of Eight Cases

Biopsy and/or autopsy material from lymphoreticular and other organs was studied in 8 children with suspected acquired immune deficiency syndrome (AIDS). One or both parents of each of these children had one or more of the recognized risk factors for AIDS, such as intravenous drug abuse, prostitution, Haitian origin. The following histologic patterns were noted in the lymph nodes: (1) follicular hyperplasia with normocellular paracortex, (2) follicular hyperplasia with depletion of paracortex, and (3) atrophy of follicles with depletion of paracortex. Lymphoid interstitial pneumonitis (LIP), a previously unreported lesion in AIDS, was present in 4 cases. It is suggested that the pulmonary lymphoid lesion may be part of a more generalized lymphoid hyperplasia involving B cells. The gross and microscopic features of the thymus, available in 2 of the 8 cases, indicated that the immunologic defect in these children was not of congenital type. Pathologic findings can be helpful in the diagnosis of the syndrome when correlated with clinical and immunologic features of suspected cases and of the pulmonary lesion. The latter is of importance in deciding the type of therapy to be given for the pulmonary disease process.     

小儿便血129例临床诊治分析

目的 探讨小儿便血的临床诊断及合理治疗的方法。方法 回顾性分析1988年1月—2001年10月收治的便血患儿129例。结果 129例患儿治愈120例，死亡8例，转儿内科治疗1例。急性坏死性小肠炎是导致死亡的主要原因。结论 小儿便血是临床常见疾病，在诊治过程中应有效应用先进的诊疗仪器，减少误诊，准确掌握手术指征，以达到较好的治疗效果。     

An Epidemic of Meningococcal Disease in Karachi (Pakistan): A Study of Children

A prospective study of 112 cases of epidemic meningococcal infection in the paediatric population is presented. The natural course of the disease, its complications, prognostic factors and therapeutic agents were studied. The cases were graded according to fixed criteria of severity which correlated well with the outcome of the disease. The gram-stain of the CSF was a sensitive and a quick method of diagnosis and crystalline penicillin a cost-effective therapy giving a good response within 48 hours in 90% of the cases.     

Safety of a fat-reduced diet: the Dietary Intervention Study in Children (DISC)

OBJECTIVE: To assess the relationship between energy intake from fat and anthropometric, biochemical, and dietary measures of nutritional adequacy and safety. DESIGN: Three-year longitudinal study of children participating in a randomized controlled trial; intervention and usual care group data pooled to assess effects of self-reported fat intake; longitudinal regression analyses of measurements at baseline, year 1, and year 3. PARTICIPANTS: Six hundred sixty-three children (362 boys and 301 girls), 8 to 10 years of age at baseline, with elevated low-density lipoprotein cholesterol, who are participants of the Dietary Intervention Study in Children. MEASURES: Energy intake from fat assessed from three 24-hour recalls at each time point was the independent variable. Outcomes were anthropometric measures (height, weight, body mass index, and sum of skinfolds), nutritional biochemical determinations (serum ferritin, zinc, retinol, albumin, beta-carotene, and vitamin E, red blood cell folate, and hemoglobin), and dietary micronutrients (vitamins A, C, E, thiamin, riboflavin, niacin, vitamins B-6, B-12, folate, calcium, iron, zinc, magnesium, and phosphorus). RESULTS: Lower fat intake was not related to anthropometric measures or serum zinc, retinol, albumin, beta-carotene, or vitamin E. Lower fat intake was related to: 1) higher levels of red blood cell folate and hemoglobin, with a trend toward higher serum ferritin; 2) higher intakes of folate, vitamin C, and vitamin A, with a trend toward higher iron intake; 3) lower intakes of calcium, zinc, magnesium, phosphorus, vitamin B-12, thiamin, niacin, and riboflavin; 4) increased risk of consuming less than two-thirds of the Recommended Dietary Allowances for calcium in girls at baseline, and zinc and vitamin E in boys and girls at all visits. CONCLUSIONS: Lower fat intakes during puberty are nutritionally adequate for growth and for maintenance of normal levels of nutritional biochemical measures, and are associated with beneficial effects on blood folate and hemoglobin. Although lower fat diets were related to lower self-reported intakes of several nutrients, no adverse effects were observed on blood biochemical measures of nutritional status. Current public health recommendations for moderately lower fat intakes in children during puberty may be followed safely.     

Massive Osteolysis of Skull Bones Secondary to Lymphangioma of Paranasal Sinuses and Mastoid Sinus: Report of Two Cases in Children

Massive osteolysis (MO) is a rare condition in which progressive localized bone tissue resorption is associated with proliferating thin-walled vessels in the absence of inflammation. Rare cases have been reported to occur in the skull. This paper describes two patients with MO who presented with massive assymetric swelling of the skull. This was associated with extensive enlargement of the paranasal sinuses (frontal, ethmoidal, and sphenoidal in one and the mastoid air cells in the other). The second patient developed subcutaneous emphysema on several occasions and the Valsalva maneuver increased the swelling, indicating transmission of the air from the nasopharynx to the mastoid cells and from there to the subcutaneous tissue. In the first patient, the sinus mucosa was shown to be involved by an extensive lymphangioma, and a similar change was seen in the mastoid air cells (patient 2). We are proposing that MO of these two patients resulted from bone resorption due to progressive extension of sinus mucous lymphangiomata. Received October 6, 2000; accepted January 31, 2001.     

Immunosuppressive Therapy of Chronic Myocarditis in Children: Three Cases and the Design of a Randomized Prospective Trial of Therapy

Three infants, each with a clinical picture of dilated cardiomyopathy, underwent endomyocardial biopsy. Immunohistologic analysis revealed chronic myocarditis. In one infant, a postviral etiology of chronic myocarditis could be assessed on the basis of molecular techniques. Therapy with azathioprine and prednisone resulted in the normalization of echocardiographic findings. Based on these observations, a randomized, multicenter treatment study of chronic myocarditis in children (TCMC) has been initiated.     

Primary Intestinal Myopathy, a Cause of Chronic Idiopathic Intestinal Pseudoobstruction Syndrome (Cips): Clinicopathological Studies of Seven Cases in Children

Clinicopathological data on seven instances of primary intestinal myopathy in children are reported. The ages of the patients ranged from eleven months to thirteen years. A persistent intestinal obstruction was the main and constant clinical feature. An ineffective intestinal propulsion was documented on manometric studies. Various urological abnormalities were present in three cases. One patient died and six survive but are dependent on enteral and parenteral nutrition. The morphological findings consisted of degenerative changes involving the muscular layers of the intestinal wall. These changes varied from cytoplasmic vacuolation to definite atrophy and disappearance of the muscular fibers. An extensive interstitial fibrosis underlined these atrophic changes in the late stages of the disease.

A familial history was identified in three cases, one consistent with an autosomal dominant transmission.     

呼吸道合胞病毒感染后期Poly(I:C)诱发小鼠气道炎症及其机制研究

目的探讨呼吸道合胞病毒(RSV)感染后再发呼吸道病毒感染时诱发气道炎症及反复喘息的致病机制。方法 64只6~8周雌性BALB/c小鼠随机分为对照组、RSV组、Poly(I:C)组及RSV+Poly(I:C)组(n=16)。收集各组肺泡灌洗液(BALF),计数BALF中细胞总数及分类计数,苏木精-伊红(HE)染色观察肺部病理损伤,检测小鼠气道反应性(AHR),ELISA法检测BALF中IFN-γ、IL-4、IL-13、基质金属蛋白酸9(MMP-9)及基质金属蛋白酶抑制物-1(TIMP-1)水平。结果 RSV+Poly(I:C)组小鼠的气道炎症细胞浸润总数及AHR较其他3组显著增高(P0.05)。RSV+Poly(I:C)组小鼠的肺组织病理损伤较对照组及RSV组加重(P0.01);BALF中MMP-9水平较其他3组明显升高(P0.05),IL-4及TIMP-1显著低于RSV组(P0.01)。结论 RSV感染后病毒再感染可能引起MMP-9/TIMP-1表达失衡,加重气道炎症反应。     

Cellular Peripheral Neural Tumors (Neurofibromas) in Children and Adolescents: A Clinicopathological and Immunohistochemical Study

Nine examples of a cellular peripheral neural tumor (CPNT) were identified in a review of 139 peripheral nerve sheath neoplasms in children, which included 60 neurofibromas and 16 malignant peripheral nerve sheath tumors. The mean age at diagnosis of these nine patients was 7 years, with six presenting in the first decade of life and four were noted at birth. The male:female ratio was 0.5. Topographically, the tumors were located in the extremities, 4; head and neck, 3; and trunk, 2. One or another stigmata of von Recklinghausen's neurofibromatosis (VRN) was present in four patients. After initial resection, seven children remained well, but two developed a recurrence; the histology was identical to the original tumor in one case but overt malignant transformation had occurred in the second. This case was the only tumor-related death in this series. The CPN as a circumscribed but nonencapsulated mass measuring 1.8-7.5 cm in greatest dimension in the subcutaneous and deep soft tissues and had a compact spindle cell pattern, occasional mitoses, and minor foci of typical neurofibroma. Immunohistochemical staining revealed vimentin expression in all seven cases, Leu-7 in six, myelin basic protein and S-100 protein in five, desmin in one, and actin in none. In contrast to neurofibroma and malignant peripheral nerve sheath tumors, CPNT tended to occur earlier, either congenitally or in the first decade, and slightly more commonly in females. The anatomical distribution and pattern of immunoreactivity were similar to neurofibroma. However, the cellularity and mitotic activity of these neoplasms were sufficiently disquieting as to raise concerns about the prognosis, and in one case, the tumor behaved in an unequivocally malignant fashion. When a peripheral neural tumor with the pathologic features described in this study is encountered, wide excision and careful clinical follow-up are recommended.