Pre- and postnatal treatment of a pulmonary sequestration presenting as a unilateral hydrothorax

We report a case of a neonatal sequestration diagnosed antenatally as a progressive left hydrothorax when ultrasound (US) was done at 30 weeks' gestation for polyhydramnios. Postnatal US and computed tomography failed to demonstrate any lesion; magnetic resonance imaging showed a spherical mass in the left chest adjacent to the left lower lobe. At the age of 4 weeks a thoracotomy was performed and the mass was excised. Pulmonary sequestrations may present with a pleural effusion and polyhydramnios as a part of non-immune hydrops fetalis. In our case hydrops was prevented by insertion of a pleuro-amniotic shunt.     

Giant inflammatory pseudotumor of the lung masquerading as pleural effusion

We report a child with a mass lesion filling the entire left chest cavity and presenting with clinical features of a left-sided pleural effusion. The mass was excised with dramatic relief of symptoms. Histopathologic examination revealed an inflammatory pseudotumour.     

Radiologic-Pathologic Conference of Children's Hospital Boston: Intraconal mass in the orbit of an infant

A 16-month-old boy presented with left exophthalmos. He was found to have an enhancing intraconal soft-tissue mass. The differential diagnosis of the mass is discussed. The lesion was proven to be a malignant ectomesenchymoma, a very unusual tumor. Received: 20 June 1996 Accepted: 30 August 1996     

Anomalous systemic arterial supply to the basal segments of the lung presenting with a murmur

A murmur was discovered on routine preschool physical examination of a 5 year and 7 month-old boy. A lower lobe lesion was detected by chest X-ray. Aortography demonstrated that a large systemic artery from the descending thoracic aorta supplied the basilar segments of the left lower lobe, which had no normal pulmonary arterial supply. A clinical diagnosis of intralobar sequestration of the lung was made, and left lower lobectomy was performed.     

Prenatal diagnosis of a nasal glioma

Nasal glioma is a rare congenital midline malformation composed of heterotopic masses of neuroglial tissue. We report a case of fetal nasal glioma diagnosed by sonography at 22 weeks’ gestation as a vascular hypoechoic mass located on the left nasal bone. Fetal MRI excluded an underlying bone defect. At birth, the lesion appeared as a reddish mass. Post natal imaging confirmed the vascularisation within the lesion with an arterial low-flow velocity and a high-resistance spectrum, consistent with a glioma. The child underwent surgery at 5 months and final diagnosis was made on pathological examination. Therefore, a vascular lesion and a clinical aspect mimicking a haemangioma should not be considered sufficient to reach the final diagnosis.     

Chagas' disease as a cause of cerebral mass in a patient with lymphoblastic leukemia in remission

We report the case of a 15-year-old female patient, native of the province of Chaco, diagnosed with lymphoblastic leukemia 2 years ago. She was currently in maintenance therapy, with good response to treatment. Twenty days before she began with headache and fever. At admission, blood cultures were negative, and brain computed tomography showed a hypodense lesion with involvement of white matter in left occipital-parietal area. A brain MRI was performed, and a hypointense left occipital lesion was observed in T1. The lesion showed peripheral gadolinium enhancing and was hyperintense on T2, with a hypodense central area surrounded by significant edema with mass effect. Chagas serology was positive, as well as the parasitemia. The patient was treated with benznidazole, with good response to therapy: a significant clinical improvement and mass reduction were observed, within the first month of treatment.     

Chondro‐osseous respiratory epithelial adenomatoid hamartoma of the nasal cavity

Chondro‐osseous respiratory epithelial adenomatoid hamartoma is a rare hamartomatous lesion of the nasal cavity, with only five cases reported in the literature to date. We report the case of a 3‐year‐old boy who presented with nasal obstruction and a mass in the left nasal cavity. The mass was completely resected on endoscopy. On microscopy, hamartomatous proliferation of respiratory‐type glands admixed with islands of immature hyaline cartilage, characteristic of chondro‐osseous respiratory epithelial adenomatoid hamartoma, was seen. Neither local recurrence nor distant metastasis was observed after 6 month follow up. Recognition of chondro‐osseous respiratory epithelial adenomatoid hamartoma as a benign lesion is important to avoid unnecessary surgical procedures.     

A case of cerebellar capillary hemangioma with multiple cysts

Intracranial capillary hemangiomas are very rare, though several spinal capillary hemangiomas have recently been reported. We report here a case of intracranial capillary hemangioma with multiple cysts and review the current literature of similar cases. A 4-month-old girl was referred to our hospital for treatment of hydrocephalus and a cerebellar mass lesion. She presented with hemangiomas distributed widely over the body, as well as disseminated hemangiomas in the pleura, liver, spleen, pancreas, kidneys and vagina. Pathological examination of the specimen from the vagina confirmed the diagnosis of a capillary hemangioma made at another hospital. Radiological examination of the brain revealed a cystic mass lesion in the left cerebellar hemisphere with subsequent obstructive hydrocephalus. The cysts extended upward into the bilateral ventricle. Following neuroendoscopic fenestration of the cysts, resection of the left cerebellar mass was performed. Histological examination of the lesion demonstrated similarly sized capillaries, and the pathological diagnosis was confirmed as capillary hemangioma. There was no recurrence postoperatively. Our patient was treated successfully by surgical resection and neuroendoscopic procedures. Surgical intervention may therefore be indicated in intracranial capillary hemangiomas.     

Multicentric osteosarcoma presenting as retrobulbar mass: a diagnostic enigma

Osteosarcoma is the most common primary malignant bone tumor of children and adolescents. It often presents as a solitary lesion; multicentric osteosarcoma with synchronous lesions occurring at multiple skeletal sites is very rare. We report a 9-year-old boy with multicentric osteosarcoma who presented with a left retrobulbar non-sclerotic mass. The multiple lesions in bone were mostly non-sclerotic on radiological examination except for a single lesion in the left tibia. Biopsy of the retrobulbar mass showed an unclassifiable poorly differentiated malignant tumor. Marrow aspiration smears showed many large, often segregated, round cells that expressed NB84a. However, trephine biopsy showed the formation of tumoral osteoid by the malignant cells, finally permitting the definitive diagnosis of osteosarcoma to be made. A hypertetraploid clone with complex structural abnormalities was demonstrated by cytogenetic study.     

Simple cyst of the testis: a rare and benign cause of testicular swelling in infancy

We report a five-month-old boy who was referred to the hospital with a presumptive diagnosis of hydrocele of the cord. Ultrasonography revealed a cystic mass within the left testis. A complete removal of the cystic mass was done. Histopathological diagnosis was simple cyst of the testis. This rare lesion should be considered in the differential diagnosis of cystic testicular lesions in infancy. The treatment of choice is simple enucleation of the cyst with sparing of the testis and related structures.     

Cystic lymphangioma of the scrotum

A 7-year-old boy who presented with a painful left hemiscrotal mass was diagnosed with acquired lymphangioma of the scrotum. Chronic friction from a cast for Perthes' disease might have been the cause of sudden enlargement of a congenital lymphangioma of the scrotum. Magnetic resonance imaging (MRI) was useful for preoperative diagnosis and determining the extent of the scrotal lesions. Total excision of the mass leaving the overlying skin was successfully performed. The clinical significance of MRI for preoperative diagnosis and planning surgical resection of this lesion is discussed. Accepted: 1 April 1997     

Intra-abdominal extralobar pulmonary sequestration

A left-upper-quadrant abdominal mass was identified in a routine ultrasound (US) examination in the 16th week of gestation. The sonographic features were those of a homogeneous, hyperechogenic lesion situated between the diaphragm and the left kidney. Other radiologic examinations (CT, MRI) confirmed the mass, but a presumptive diagnosis could not be made. The lesion was excised and histologic examination demonstrated an extralobar pulmonary sequestration. Although it is an uncommon type of congenital malformation, pulmonary sequestration should be included in the differential diagnosis when an echogenic intraabdominal mass is detected on antenatal US.     

Duplication cyst of the stomach presenting as hemoptysis.

We report a rare case of a 2-year-old boy with a gastric duplication cyst located in the stomach, presenting with vague abdominal pain, recurrent cough, hemoptysis and a lesion in the left lower lobe of the lung reported as a sequestration. The duplication cyst was attached to the diaphragm and lungs by a narrow tract. The cyst was excised. Subsequent scans of the chest showed regression of the lung lesion. We believe this to be the first case in the literature where the hemoptysis was cured and the pulmonary lesion regressed completely after excision of the duplication cyst of the stomach alone. Pulmonary resection was thus avoided.     

Primary intradural mesenchymal chondrosarcoma of the spine in a child

We report a primary intradural mesenchymal chondrosarcoma of the spine in a 3-year-old girl. MRI revealed a markedly enhancing oval mass associated with focal areas of low signal intensity extending from T11 to L1. The lesion was located posterolateral to the right side of the spinal cord, pushing the conus medullaris and cauda equina anteriorly and to the left. The adjacent spinal cord also showed serpiginous areas of flow void. The mass was completely removed. Microscopic examination and immunohistochemical studies confirmed the diagnosis of mesenchymal chondrosarcoma. The patient was free of symptoms after surgery.     

Pigmented free-floating vitreous cyst in a child

An 8-year-old asymptomatic boy was referred for a pigmented fundus lesion in his left eye that was suspected to be a possible choroidal melanoma. Visual acuity was 20/20 in each eye. Ocular examination in the upright position revealed a pigmented lesion measuring 4 mm in diameter and 3 mm in thickness located inferior to the fovea. Following supine positioning, the mass floated to the superior macular region. UI-trasonography confirmed a cystic vitreous mass. Observation of the benign vitreous cyst was advised. A free-floating pigmented cyst can resemble a pigmented intraocular tumor, particularly if it is immediately preretinal.     

Localized sarcoma of the chest wall

This 9-year-old white male suffered mild trauma to the left side of the chest on two occasions in April 1981, and then developed a low-grade fever. A chest x-ray film showed pneumonia. He was then given oral ampicillin for 2 weeks, and the fever and left chest pain resolved. Repeat chest radiograph one month later showed a persistent density, and a bone scan revealed increased uptake in the left fifth rib. Osteomyelitis was diagnosed, and intravenous antibiotics were administered. In June 1981, the density had become larger on radiographic examinations, and a left chest wall mass was noted for the first time. He was referred to the Children's Hospital of Philadelphia, with the suspected diagnosis of Ewing's sarcoma of the left fifth rib. On arrival at the Children's Hospital of Philadelphia on June 13, 1981, the physical examination was unremarkable except for a 1 X 2-cm mass overlying the fifth rib anterolaterally. Dr Hugh Watts of the Division of Orthopedic Surgery reviewed the radiographs and bone scan and felt that the appearance was not consistent with primary Ewing's sarcoma of the rib. The boy was then taken to surgery by Dr. Moritz Ziegler on June 23, 1981, at which time biopsy of the lesion was accomplished. The tumor had by then grown to a size of 3 X 5 cm.     

Massive Congenital Mesenchymal Malformation of the Lung: A Case Report with Ultrastructural Study

An unusual congenital tumorous malformation of mesenchymal tissue caused severe respiratory distress in a premature infant. The solid mass, which replaced almost the entire left lung, was composed of interlacing fascicles of spindle cells that percolated through otherwise well-developed fetal lung tissue. The normal anatomic arrangement of bronchial components was altered, but bronchial cartilages were present. Ultrastructural study of the lesion showed mesenchymal cells with fibroblastic differentation.     

Multiple pilomatrixomas affecting the eyelid and face

A 3-year-old girl presented with a right upper eyelid mass. The lesion had a reddish appearance, was firm to palpation, and was fixed to underlying tissues. Fourteen months after the lesion was excised, a similar lesion was discovered on the left side of the nose and was also excised. Histopathologic examination of the excised tumors revealed variable basophilic hair matrix cells and sheets of nonviable eosinophilic shadow cells. Foci of dystrophic calcification were also seen in the necrotic tumor areas. The histopathologic findings were found to be consistent with pilomatrixoma. Results of limited clinical work-up of the child for Gardner's syndrome, sarcoidosis, and myotonic dystrophy were negative. Multiple periocular and facial pilomatrixomas can occur in children in the clinical absence of myotonic dystrophy, Gardner's syndrome, and sarcoidosis.     

Massive congenital mesenchymal malformation of the lung: a case report with ultrastructural study

An unusual congenital tumorous malformation of mesenchymal tissue caused severe respiratory distress in a premature infant. The solid mass, which replaced almost the entire left lung, was composed of interlacing fascicles of spindle cells that percolated through otherwise well-developed fetal lung tissue. The normal anatomic arrangement of bronchial components was altered, but bronchial cartilages were present. Ultrastructural study of the lesion showed mesenchymal cells with fibroblastic differentation.     

Mesectodermal leiomyoma of the ciliary body: new evidence for neural crest origin

An 8-year-old white boy had a mass of the ciliary body in his left eye. Both the translucent characteristics and the magnetic resonance imaging findings suggested that this lesion was cystic. Fluid aspiration biopsy and iridocyclectomy were performed. By light microscopy the neoplasm showed the characteristic appearance of a neuroid tumor; however, on electron microscopic examination, the tumor exhibited characteristic features of a smooth muscle neoplasm and was diagnosed as a mesectodermal leiomyoma. Observation of melanin granules in scattered tumor cells further confirmed that the tumor had the same origin as uveal melanocytes, which also derive from the neural crest.