儿童鼻型NK/T细胞淋巴瘤1例并文献复习

目的 报道罕见儿童鼻型NK/T细胞淋巴瘤1例。方法 总结分析儿童鼻型NK/T细胞淋巴瘤1例的临床及组织病理学特征。详细采集患儿体格检查、鼻窦CT和MRI、骨髓像、免疫学及组织病理学检查等资料,并进行文献复习。结果 患儿,男,15岁,因“发热、皮疹及鼻堵1个月”入院。 入院查体可见全身散在大小不等皮疹,略高出皮肤,直径1～5 cm,呈鲜红色及紫红色,以双下肢多见,左下肢胫骨内侧可见一直径约7 cm皮肤结节。鼻翼肿胀,鼻腔内有增生物,有异味及黄色分泌物。鼻窦CT及MRI均提示鼻腔占位性病变。患儿入院后腹痛进行性加重,腹部X线检查提示肠穿孔,行直肠穿孔修补、阑尾切除及回肠提吊造瘘术。左下肢皮疹病理学检查示：真皮小血管及皮肤附件周围可见异形淋巴细胞浸润;左下肢胫骨内侧皮肤结节穿刺涂片见大量肿瘤细胞;切除肠管病理学检查示：部分盲肠壁黏膜及肌间可见灶状异形淋巴细胞浸润,其形态与左下肢皮疹病理学检查中所见相似。免疫组化示：CD45RO(++),CD3(++)~（+++), CD56(++)~（+++);GranzymeB、CD20、CD79a和CD68均(-)。结合临床表现、病理学检查镜下形态及免疫组化结果,确诊为鼻型NK/T细胞淋巴瘤。患儿家属放弃治疗,患儿于出院2个月后死亡。结论 鼻型NK/T细胞淋巴瘤主要累及鼻腔及面中线部位,并可表现为多系统受累。患儿如有呈进行性面部中线破坏性病变应高度警惕本病的可能。     

Teratoma Associated with Endodermal Sinus Tumor

A 3-1/2-year-old girl was diagnosed as having a mediastinal endodermal sinus tumor with pulmonary, bony, and hilar lymph node metastases. Following 7 months of treatment with chemotherapy, thoracic CT (computerized tomogram) scan showed the presence of a residual mass. Upon further surgical exploration the residual mass, which was found to be intrapericardial and arising from the ascending aorta, was resected. This second lesion was shown to be a benign cystic teratoma.

The combination of an intrapericardial teratoma and a mediastinal endodermal sinus tumor in a female child is probably unique. The possible etiology for the combination of these two pathologies is discussed.     

Nasal chondromesenchymal hamartoma of infancy clinically mimicking meningoencephalocele

We report a typical case of nasal chondromesenchymal hamartoma (NCMH) of infancy, which extended to the orbit and intracranium through the skull base. The nasal mass was incidentally found in a 5-month-old otherwise healthy boy who had recently developed ptosis of the left eye. On neuroimaging, there were defects in the left ethmoid bone of the anterior cranial fossa and medial orbital wall. A round heterogeneous mass was found in the left nasal cavity. Solid portions were strongly enhanced. The preoperative diagnosis was nasal glioma or encephalocele. After frontal craniotomy, the mass was separated from the cranial basal dura. Through the transnasal approach, the mass was dissected from the left orbital content and removed. NCMH is a rare benign lesion and histopathological analogue of the mesenchymal hamartoma of the chest wall, having unique pathology: islands of hyaline cartilage, somewhat calcified or ossified hyalinized collagenous trabeculae and abundant myxoid stroma. Its occurrence in the nasal cavity was identified in 1998. The recognition of this entity is necessary to avoid misdiagnosis of the frozen section and for appropriate surgery.     

Intraorbital and intracranial soft-tissue glomus tumor in an 8-year-old child. Case report

Soft-tissue glomus tumors (or glomangiomas) are unrelated to neuroendocrine paragangliomas (glomus tympanicum, jugulare, and vagale). The authors present the first reported case of an orbital soft-tissue glomus tumor in a child. An 8-year-old girl developed rapidly progressive right-eye blindness, proptosis, and a sixth cranial nerve palsy. Magnetic resonance imaging demonstrated a homogeneously enhancing lesion extending from the right orbit through the superior orbital fissure to the cavernous sinus and middle cranial fossa. A biopsy specimen demonstrated the lesion to be a soft-tissue glomus tumor. Following angiography and embolization, a gross-total resection of the tumor was achieved. The patient was treated with adjuvant proton-beam radiotherapy. At 24 months follow-up her proptosis and sixth cranial nerve palsy had resolved and there was no evidence of tumor recurrence.     

Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging

The newborn, infant, or young child who presents with a midline frontonasal mass often poses a diagnostic challenge to the clinician. The most pressing issue is whether the mass extends intracranially. The development of the frontonasal region or anterior neuropore is complex. Aberrant embryogenesis leads to three main types of anomalies: nasal dermal sinus, anterior cephalocele, and nasal glioma. Understanding the developmental anatomy of the anterior neuropore and postnatal maturation will serve the radiologist well when it comes to imaging frontonasal masses. Pitfalls particularly common to CT imaging interpretation include the evolving ossification of the frontal, nasal and ethmoid bones in the first year of life, morphology and size of the foramen cecum, and the natural intumescence of the anterior nasal septum. Determination of the presence of a connection between the frontonasal mass and the anterior cranial fossae is crucial in the imaging assessment and clinical management. In the case of the nasal dermal sinus, failure to appreciate the intracranial components of the malformation can lead to fatal meningitis. MR imaging is the modality of choice for assessing the pediatric frontonasal region. Its advantages include multiplanar imaging, distinguishing the interface among cartilage, bone, brain and fluid, diffusion imaging to detect epidermoid tumors, and the capacity to evaluate the brain for associated cerebral anomalies.     

Intracranial manifestation of osteosarcoma

Involvement of the central nervous system in osteosarcoma is uncommond. These neoplasms are most often located at the metaphyses of tubular bones and rarely in flat bones of vertebra, ribs, pelvis, facial bones, or skull. Tumors of the latter bones may obviously spread into the cerebrum. Osteosarcomas primarily metastasize hematogenously to the lungs. Bone, lymph node, or brain metastases are mostly seen following or concomitantly with pulmonary metastatic disease. However, there are single cases of primary osteosarcoma of the brain parenchyma without bone association or tumor manifestation at other locations. Three illustrative cases highlight the diversity of the clinical presentation of cerebral osteosarcoma: a 22-year-old man with multiple brain metastases following late pulmonary relapse of an osteosarcoma of the tibia, a 31-year-old woman with an osteosarcoma of the left anterior cranial fossa arising from the skull base, and a 78-year-old man presenting with primary osteogenic sarcoma of theleft frontal cerebral hemisphere. According to the current literature, 10-15% of all osteosarcoma patients experiencing relapse may beat risk for central nervous system metastases. To the authors' best knowledge, there are 11 cases of primary intracerebral or meningeal osteogenic sarcoma, including this case report, without any skeletal attachment.     

A large planum sphenoidale meningioma with sinonasal extension in a child. Case report and review of the literature

Anterior cranial base meningiomas are rare tumors in children. Due to the extensive involvement of orbit, paranasal sinuses, midface, and anterior skull base, a multidisciplinary approach is warranted. We present a case of a child with a large planum sphenoidale meningioma extending into subfrontal region, ethmoid and maxillary sinuses inferiorly, and orbits laterally. The patient, a 4-year-old girl, presented with long-standing nasal stuffiness and swelling of the midface. An extended frontobasal approach through a bifrontal craniotomy was used to resect the intracranial portion of this mass. The tumor had eroded through nasal septum, medial orbital walls, and left maxilla, structures which were not readily accessible from a cranial approach alone. A modified Weber-Ferguson incision was used for a transfacial approach to resect the residual mass below the skull base. The advantages of combining the bifrontal craniotomy with a transfacial split provided the added exposure to maximize the extent of resection.     

INTRACRANIAL MANIFESTATION OF OSTEOSARCOMA

Involvement of the central nervous system in osteosarcoma is uncommond. These neoplasms are most often located at the metaphyses of tubular bones and rarely in flat bones of vertebra, ribs, pelvis, facial bones, or skull. Tumors of the latter bones may obviously spread into the cerebrum. Osteosarcomas primarily metastasize hematogenously to the lungs. Bone, lymph node, or brain metastases are mostly seen following or concomitantly with pulmonary metastatic disease. However, there are single cases of primary osteosarcoma of the brain parenchyma without bone association or tumor manifestation at other locations. Three illustrative cases highlight the diversity of the clinical presentation of cerebral osteosarcoma: a 22-year-old man with multiple brain metastases following late pulmonary relapse of an osteosarcoma of the tibia, a 31-year-old woman with an osteosarcoma of the left anterior cranial fossa arising from the skull base, and a 78-year-old man presenting with primary osteogenic sarcoma of theleft frontal cerebral hemisphere. According to the current literature, 10-15% of all osteosarcoma patients experiencing relapse may beat risk for central nervous system metastases. To the authors' best knowledge, there are 11 cases of primary intracerebral or meningeal osteogenic sarcoma, including this case report, without any skeletal attachment.     

Intracranial complications following mastoidectomy

Mastoidectomy is a common surgical procedure in otology. However, postoperative complications of various degrees of severity may occur. We present 4 children who underwent mastoidectomy for middle ear and mastoid disease and developed postoperative intracranial complications. One child was operated on for brain abscess 1 week after the initial mastoidectomy. Another child appeared with seizures 5 days after the initial mastoidectomy and a subdural empyema was drained during revision surgery. Large bone defects with exposed middle cranial fossa dura were found at revision surgery in both cases and Proteus vulgaris and methicillin-resistant Staphylococcus aureus were isolated from the mastoid and abscess cavities in these children. A small epidural collection was diagnosed in the third patient 2 days after initial mastoid surgery and was managed with intravenous antibiotics only. The other child was found to have sigmoid sinus thrombosis the day after mastoidectomy that was performed for nonresponsive acute mastoiditis. This child received both intravenous antibiotics and anticoagulants. Timely revision surgery, combinations of third- or fourth-generation cephalosporins with vancomycin or metronidazole and the addition of anticoagulants in cases of sinus thrombosis can lead to full recovery.     

CT demonstration of asymptomatic pulmonary emboli after bone marrow transplantation: case report

Asymptomatic pulmonary emboli producing microcalcifications were observed 200 days after bone marrow transplantation on a routine surveillance CT in a child. The findings consisted of peripheral hazy, nodular infiltrates in the lower lung fields. To our knowledge this has not been recorded in the radiologic literature.     

Fibromyxoid sarcoma in a four-year-old child: Case report and review of the literature

We present a child with a rare and chemotherapy-resistant form of soft-tissue cancer, low-grade fibromyxoid sarcoma, first noted when he was 4 years old. He is the youngest patient reported to date. An 11-year-old white male presented to The University of Texas M. D. Anderson Cancer Center's Department of Pediatrics with a 7-year history of right thigh mass and pulmonary nodules, confirmed on examination. He had undergone extensive prior chemotherapy and surgery. He received chemotherapy with high-dose cyclophosphamide (7 g/m²) and later etoposide (150 mg/m²/day × 5), with only slight shrinkage of the thigh mass and none in the lungs. Subsequently the tumor in his proximal thigh and his lung metastases were resected, and radiation therapy was administered to the thigh. His disease remained stable for 12 months, but he then developed a pleural-based metastasis on the left side and new, bilateral lung metastases also. The tumors on the left side were removed; residual disease is stable after treatment for 6 months with subcutaneous alpha-interferon-2b. Low-grade fibromyxoid sarcoma is very uncommon in children. It grows slowly and metastasizes to distant organs, chiefly to the lungs. It is resistant to conventional chemotherapy, and thus far only surgery seems to have a life-prolonging effect. Newer chemotherapeutic and possibly biologic agents should be tried in future patients, in order to find an effective way to control the disease. © 1996 Wiley-Liss, Inc.     

Atypical meningioma and extensive calvarium defects in neurofibromatosis type 1

A 9-year-old girl with neurofibromatosis type 1 (NF1) presented with a massive atypical meningioma and calvarial defect. Skull radiographs and cranial CT showed an extensive lytic bone lesion at the vertex. MRI demonstrated a large mass invading the calvarium and sagittal sinus. The histopathological and immunohistochemical diagnosis of the resected mass was atypical meningioma. To our knowledge, this is the first case of NF1 associated with atypical meningioma and massive calvarial defect in a child.     

Treatment of metastatic sialoblastoma with chemotherapy and surgery

Tumors of the salivary gland are very uncommon in children. Sialoblastoma is a rare, aggressive, blastomatous, and potentially malignant congenital tumor. Distant metastases are rare. We present a case of sialoblastoma with lung metastases that developed in a 4-year-old girl adjacent to a congenital nevus in the left cheek. The tumor was inoperable at diagnosis but the largest of the pulmonary metastases was removed surgically. The patient responded well to chemotherapy and underwent surgical excision of the primary tumor, followed by three more courses of chemotherapy.     

Generation of patient‐derived tumor xenografts from percutaneous tumor biopsies in children with bone sarcomas

One of the limitations of performing percutaneous biopsies in patients with bone sarcomas is the small amount of tumor that can be obtained for research purposes. Here, we describe our experience developing patient‐derived tumor xenografts (PDXs) using percutaneous tumor biopsies in children with bone sarcomas. We generated 14 bone sarcoma PDXs from percutaneous tumor biopsies. We also developed eight bone sarcoma PDXs from surgical resection of primary bone tumors and pulmonary metastases. A multidisciplinary team approach was critical to establish an accurate diagnosis and to provide adequate tumor samples for PDX generation.     

Left Coronary Artery Arising from the Non-Coronary Sinus: A Rare Congenital Coronary Anomaly

Anomalous origin of the left coronary artery is a cause of sudden death in adolescents and young adults. Most commonly, it originates from the right coronary sinus or the pulmonary artery. Origin of the left main coronary artery from the non-coronary sinus of Valsalva is extremely rare. We report a case of a child with anomalous origin of the left main coronary artery from the non-coronary sinus diagnosed during the evaluation of a ventricular arrhythmia.     

Pulmonary nodules secondary to total parenteral alimentation

A seven-year-old male, who had a retroperitoneal alveolar rhabdomyosarcoma and was on total parenteral alimentation (TPN) developed multiple pulmonary nodules, indistinguishable from metastases. These proved to be multiple lipid emboli on open biopsy.     

Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature

A 2 1/2-year-old female with a sphenooccipital-vertebral chordoma presented with neck pain, torticollis, fever, a lytic lesion of C2 vertebra, and bilateral nodular infiltrates in the lung. The lung biopsy revealed multiple tumor emboli by an enigmatic epithelioid-appearing neoplasm with immunohistochemical staining for vimentin, cytokeratin, and epithelial membrane antigen. A thorough roentgenographic evaluation disclosed a destructive, prepontine mass in the region of the clivus, erosion of the odontoid process, and compression of the cervical spinal cord. The patient died after a clinical course of 3 months. We identified 16 additional cases of metastasizing chordomas in the pediatric-age population; this case is the first to our knowledge with pathologically documented pulmonary metastasis at presentation.     

Fatal Paradoxical Embolism to the Left Carotid Artery During Partial Resection of Wilms' Tumor

An 8-year-old boy with an uncorrected ventricular septal defect, pulmonary stenosis, mental retardation, and gigantism died 24 hours after partial resection of a large right-sided Wilms' tumor. The presence of other abnormalities, including a small umbilical hernia and overgrowth of the external genitalia, raises the possibility that this case represents a variant of the Beckwith- Wiedemann syndrome. The typical facial features of Sotos' syndrome were not present. Gross examination of the surgical specimen revealed that tumor was present in the resected margin of left renal vein. Necropsy showed that death resulted from extensive cerebral infarction due to occlusion of the left internal carotid artery and its branches by tumor emboli Paradoxical embolism had occurred during or soon after partial resection of the tumor mass due to passage of tumor fragments into the systemic circulation through the ventricular septal defect.     

The pivotal role of deep vein thrombophlebitis in the development of acute disseminated staphylococcal disease in children

Deep vein thrombophlebitis (DVT) and septic pulmonary emboli (PE) are rare in children. The association of DVT and acute disseminated staphylococcal disease (DSD) during childhood has not been previously reported. We report 3 children who developed a triad of DVT, septic PE, and acute osteomyelitis with Staphylococcus aureus cultured from blood and bone. One child succumbed, while 2 survived following prolonged, morbid hospitalizations. The rapid clinical deterioration observed in these patients might be caused by the aggressiveness of staphylococcal infection combined with an ongoing showering of septic emboli from the ileo-femoral DVT. We suggest that infected DVT with septic PE had a pivotal role in the development of DSD in these children. The presence of this triad should prompt aggressive treatment with the appropriate antibiotics, anticoagulation, surgical drainage, and assisted ventilation when indicated.     

Congenital peripheral primitive neuroectodermal tumor refractory to treatment

The authors report an unusual case of an infant presenting with a congenital peripheral primitive neuroectodermal tumor (PPNET) of the left hand refractory to treatment. A newborn girl was born with a large bluish-red mass of 4.5 cm diameter protruding into the palm and the dorsum of the left hand. Tumor biopsy confirmed the diagnosis of PPNET. The initial metastatic workup for the detection of metastases was negative. Four cycles of chemotherapy according to CCSG 7881/POG 8850 regimen B were given. Despite this aggressive chemotherapy the tumor grew to involve the entire left hand. The left hand was amputated, and then two cycles of topotecan/cyclophosphamide were given. Five months later extensive metastases developed, involving the brain, lungs, liver, and skeleton, and the child died at the age of 2.5 years. PPNET presenting at birth is uncommon; presentation in the hand is unusual, and the fact that it did not respond to treatment is still more uncommon.