What is orbital pseudotumor

Idiopathic pulmonary haemosiderosis is a rare cause of diffuse alveolar haemorrhage of unknown aetiology. It occurs most frequently in children, has a variable natural history with repetitive episodes of diffuse alveolar haemorrhage, and has been reported to have a high mortality. Many patients develop iron deficiency anaemia secondary to deposition of haemosiderin iron in the alveoli. Examination of sputum and bronchoalveolar lavage fluid can disclose haemosiderin-laden alveolar macrophages (siderophages), and the lung biopsy shows numerous siderophages in the alveoli, without any evidence of pulmonary vasculitis, nonspecific/granulomatous inflammation, or deposition of immunoglobulins. Contrary to earlier reports, corticosteroids alone or in combination with other immunosuppressive agents may be effective for either exacerbations or maintenance therapy of idiopathic pulmonary haemosiderosis.

https://erj.ersjournals.com/content/erj/24/1/162.full.pdf

Idiopathic pulmonary haemosiderosis revisited

Two cases of melanotic neuroectodermal tumor of infancy were examined by electron microscopy and an extensive review of the literature was performed. Ultrastructural examination revealed several features not previously described for this lesion. Three different types of melanin granule formation were observed, many cells had a single cilium, and cell junctions of the "close" or "modified-tight" type were seen, but no desmosomes were found. The morphologic data were consistent with the concept that the tumor arises from neural crest cells. The review of the literature indicated that there have been 158 reported cases of this lesion, including the two reported here. There were five cases of malignant tumor reported for a rate of 3.2%. This rate of malignancy is very high in view of the fact that the tumor has been described as universally benign. The significance of this finding is discussed with regard to melanin-producing lesions of the oral cavity.

Melanotic neuroectodermal tumor of infancy: An ultrastructural study, literature review, and reevaluation

Choristomas of the oral cavity: a review.

Prognosis in pediatric idiopathic pulmonary hemosiderosis.

• This report describes the clinical features and pathologic anatomy seen in an infant with anomalous bronchial branching and other congenital anomalies. The patient died of respiratory insufficiency and infection, possibly fostered by the malformed airway and a coexistent partial anomalous venous return. At autopsy, bronchial drainage of the right middle and lower lobes was provided by a large bronchial branch that originated in the left main-stem bronchus and bridged the mediastinum before entering the contralateral lung. Since crossed anatomic airway relationships after formation of primary branches are incompatible with current concepts of embryologic lung development, a brief critical review of the pertinent literature is undertaken. ( Am J Dis Child 130:1015-1018, 1976)

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=peds&articleId=506645&pdfFileName=archpedi_130_9_019.pdf

"Bridging bronchus". A previously undescribed airway anomaly.

An intracardiac tumor arising in the right atrium of a young child is reported. Morphologically, the lesion was reminiscent of “plasma cell granulomas,” but unprecedented in this location. Based on clinical manifestations, laboratory findings, histologic and ultrastructural characteristics of the mass, the speculation is put forth that this lesion may represent a cardiac myxoma with atypical structural features. An extension of this proposal is a pathogenetic sequence that would try to reconcile the complex clinical course of some myxomas with the described structural observations. Alternative choices for classification of the lesion are also given consideration.

Unusual intracardiac tumor in a child: Inflammatory pseudotumor or “granulomatous” variant of myxoma?

A case is described of heterotopic brain tissue with simultaneous occurrence of melanotic neuroectodermal tumor in the oropharynx of a 6-week-old infant. The melanotic neuroectodermal tumor was embedded within the heterotopic glial tissue. This coexistence leads us to speculate that a defect causing a pinching-off of both neural crest cap and medullary epithelium of neural tube might have taken place at, or before, the 25-30 somite stage. The displaced embryonic structures subsequently differentiated into glial tissue, choroid plexus, and melanotic neuroectodermal tumor. This observation may be interpreted as further support in favor of a neural crest origin of juvenile melanotic neuroectodermal tumor.

https://acsjournals.onlinelibrary.wiley.com/doi/epdf/10.1002/1097-0142%28197607%2938%3A1%3C249%3A%3AAID-CNCR2820380137%3E3.0.CO%3B2-D

Simultaneous occurrence of melanotic neuroectodermal tumor and brain heterotopia in the oropharynx.

Electron microscopic examination of a lnng biopsy obtained from a child in the active stage of idivpathic pulmonary hemosiderosis disclosed prominent changes in the basement membrane of alveolar capillaries. Review of the pertinent literature showed that a stl uctural defect of lung cap­ illary walls in this disease has been proposed in the past main! y on theoretic grounds but not ade­ quately documented at the ultrastructural level. Concentric multila)ering of pulmonary capillary basal lamina, one of the changes observed in our material, is a rare phenomenon in this location, although one that has been well described in extrapuhnonary microcirculatory beds and is prob­ ably a nonspecific reaction to vascular injurv. The conceptual difficulties attending a definition of idiopathic pulmonary hemosiderosis ate briefly discussed.

Idiopathic pulmonary hemosiderosis: evidence of capillary basement membrane abnormality.

• We report an instance of splenic heterotopia arising within the left kidney of an otherwise normal female infant. Embryologic considerations suggest that fusion of nephrogenic mesoderm and splenic anlage in the second month of gestation accounts for the observed findings. Although splenic-mesonephric (splenogonadal) fusion is a well-characterized, although rare, malformation, we were unable to find any previous description of splenic-metanephric fusion. ( Am J Dis Child 131:994-996, 1977)

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=PEDS&articleId=507663&pdfFileName=archpedi_131_9_009.pdf

F. Gonzalez-Crussi

Papers

"Bridging bronchus". A previously undescribed airway anomaly.

Unusual intracardiac tumor in a child: Inflammatory pseudotumor or “granulomatous” variant of myxoma?

Simultaneous occurrence of melanotic neuroectodermal tumor and brain heterotopia in the oropharynx.

Idiopathic pulmonary hemosiderosis: evidence of capillary basement membrane abnormality.

Splenorenal fusion: heterotopia simulating a primary renal neoplasm.