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A case of olfactory neuroblastoma occurring in a 3-year-old girl is reported. The rarity of the lesion in early childhood is stressed and discussed with the clinico-pathological characteristics of the tumour, which are apparently more aggressive the younger the patient. Olfactory neuroblastoma should be considered as a possible diagnosis regardless of the age of the patient.
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In the right eye of a 2 1/2-year-old boy a markedly prominent tumor, arising from the posterior pole, was found. The clinical diagnosis was retinoblastoma and the eye was removed. Histologically, however, this appeared to be a medulloepithelioma originating in the optic disc and in the posterior retina: an extremely rare localization as these tumors usually originate in the ciliary region. The tumor mainly consisted of neuroectodermal elements in several degrees of differentiation giving the aspect of a benign (hamartomatous) embryonal brain tumor rather than those of a retinal tumor. Some aspects of the nomenclature are discussed.
A 50-year-old man presented with a reddish, well-vascularized mass in the region of a nasal iridodialysis of his left eye, pushing the whole iris in this sector forwards; the mass had a pinkish, partly cystic appearance in the pupil. The tumour was transparent on diaphanoscopy. A-scan sonography revealed a solid tumour of the ciliary body with partial retinal detachment suggesting a malignant melanoma. Fluorescein biomicroscopy did not confirm this suspicion. The enucleated eye demonstrated a malignant teratoid medulloepithelioma of the whole nasal and lower ciliary body, infiltrating the deep iris stroma, the anterior choroid, the neighbouring vitreous body and spreading along the inner retinal surface back to the equator of the eye. The occurrence of medulloepithelioma in adult and senior persons is rather exceptional and this is the reason for this publication.
A 29-year-old male patient with a microphthalmos on the right side since birth was seen in June 1987 in the University Eye Clinic because of a massive exophthalmus of the former microphthalmic eye. Computer tomography revealed a diffuse, infiltrating tumor of the right orbit. The bulbus was not demarcated, and the whole orbita had to be eviscerated. Histologically, we found a solid, undifferentiated tumor with clear signs of malignancy. It consisted of epithelial cells, partly in nest formation, which were keratin positive and vimentin negative, and of more spindle-shaped formations which were keratin negative and vimentin positive. In some areas, the tumor showed a tubular or an adenoid proliferation. CEA or S-100 could not be demonstrated. The histopathological diagnosis was neuroepithelial adenocarcinoma of the eye. The differential diagnosis and the genesis of the tumor are discussed.
BACKGROUND: Primitive neurocetodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectrodermal origin most often presenting as bone or soft tissue masses. There are very few reported cases of PNET of the kidney and none diagnosed by fine needle aspiration cytology (FNAC), to the best of our knowledge, in the world literature. We present two cases of renal PNET diagnosed on cytology. CASES: Two patients with renal masses were diagnosed as having PNET on FNAC. Cytologically the tumors showed a dispersed population of malignant small round cells with focal rosette formation and perivascular arrangement of tumor cells. Immunohistochemistry on the cell blocks in both cases showed strong membrane positivity for CD99 (MIC2). Cytogenetic studies in both cases showed the characteristic t(11;22)(q24;q12) translocation, with additional chromosomal abnormalities in case 2. CONCLUSION: PNET of the kidney is a distinct entity and can be diagnosed on fine needle aspiration smears and confirmed with immunohistochemistry and cytogenetic studies. A diagnosis of PNET must be included in the differential diagnosis of renal masses in adolescents and young adults.
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This is a report of an olfactory neuroepithelioma occurring in a male cynomolgus monkey (Macaca fascicularis). It is not possible to state whether this tumor was spontaneous or related to the experimental procedures carried out in this animal.
Expression of insulin-like growth factor I (IGF-I) mRNA by some tumor cell lines of neuroectodermal origin has been described. To further explore the significance of IGF-I mRNA expression in these tumors, a more extensive analysis was performed. Most (9 of 10) neuroectodermal tumor cell lines with a t(11;22) translocation (primitive neuroectodermal tumor [PNET], Ewing's sarcoma, esthesioneuroblastoma) expressed IGF-I mRNA, whereas 0 of 15 cell lines without the translocation (PNET, neuroblastoma) expressed IGF-I. Furthermore, inasmuch as all neuroblastoma (12 of 12) cell lines examined expressed IGF-II RNA, the pattern of IGF expression could distinguish between these closely related tumors. CHP-100, a PNET cell line with the t(11;22) translocation, was shown to secrete both IGF-I protein and an IGF binding protein, IGFBP-2. This cell line also expressed the type I IGF receptor mRNA, and blockade of this receptor by a monoclonal antibody (alpha IR3) inhibited serum-free growth. These data demonstrate that IGF-I expression is a property of neuroectodermal tumors with a t(11;22) translocation and that interruption of an IGF-I autocrine loop inhibits the growth of these tumor cells.
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Esthesioneuroblastoma is a nasal tumor which arises from cells of neural crest origin. It is a difficult tumor to diagnose clinically and histopathologically. First described in 1924, approximately 160 cases have been reported with over 125 of these in the last 15 years. This reflects an increased awareness of the tumor by physicians rather than an icreased incidence. In the past 17 years, 12 cases of esthesioneuroblastoma have been treated at the Department of Otolaryngology and Maxillofacial Surgery of the University of Virginia Medical Center. Reviewing these cases and the literature leads us to make the following recommendations for diagnosis and treatment: The diagnosis of esthesioneuroblastoma can be made by 1) the clinician who suspects it in any patient with a nasal mass causing unilateral obstruction; 2) the finding of plexiform intercellular fibrils by light microscopy (rosettes and pseudorosettes are not as common as reported); 3) the finding of secretory granules and neurites by electron microscopy of the highly undifferentiated tumors; and 4) formaldehyde-fume-induced fluorescence. Combined therapy with preoperative irradiation followed by craniofacial resection of the tumor to include the cribriform plate is recommended. This treatment should result in a five-year survival in excess of 50% of patients.
Tumors of the olfactory epithelium of rats were induced with two different nitrosamines: 2,6-dimethylnitrosomorpholine and N-nitrosopiperidine. Both carcinogens yielded identical tumors consisting of small, undifferentiated, neuroblastic cell elements without specialized cell contact. Cell processes contained microtubuli, centrioles, and neurosecretory granules. Two kinds of rosettes were encountered frequently: neuroblastic Homer Wright rosettes consisted of undifferentiated cells, surrounding a minute lumen filled with amorphous material; and Flexner rosettes showed a higher degree of maturation. Inside their central lumen, cell processes with characteristic features of olfactory sensory cells (basal bodies, cilia, centrioles, microtubuli) could be demonstrated. The stem cell of this tumor is most likely the undifferentiated light basal cell inside the olfactory epithelium, since its ultrastructural appearance and its cytoskeleton are alike. At least under neoplastic conditions, this stem cell may likewise differentiate into epithelial cells, since transition to squamous cell carcinomas has been observed. In view of their overwhelming similarity to their human counterpart, the induced tumors are most likely to represent esthesioneuroepitheliomas.
For the period 1974 to 1988, 148 patients undergoing 162 rhinotomies at The Mount Sinai Medical Center, New York, for a variety of benign and malignant neoplasms were reviewed with particular attention to postoperative complications. The effects of certain predisposing factors such as prior rhinotomy, lateral versus total rhinotomy, additional medial maxillectomy or craniofacial resection, and preoperative irradiation or nasal bone erosion are discussed. In addition, a surgical technique of rhinotomy with medial maxillectomy is presented that includes maneuvers designed to prevent these complications.
Olfactory neuroblastoma is an uncommon malignant tumor arising from olfactory epithelium. Generally considered to be a tumor of slow growth, it varies in aggressiveness and may cause death by distant metastasis or local invasion. The pathologic diagnosis is difficult, and this tumor is often erroneously diagnosed. Nine previously unreported cases are presented, and a review of the various treatment modalities and course of the disease is given. Maximum therapeutic succes is best achieved by radical excision followed by a full course of radiation. Because of the high incidence of local recurrence (46%) and slow growth, patients should be followed up closely for life.
While magnetic resonance imaging (MRI) is no longer a tool of the future, its availability is limited. Most hospitals still use high-resolution computed tomography (CT) scanning as their major imaging modality. Although numerous articles point to the improved diagnostic uses of MRI--as compared to high-resolution CT--a change from high-resolution CT scanning to MRI scanning throughout the United States would cost billions of dollars. We should, therefore, delineate those clinical situations in which magnetic resonance imaging is distinctly superior to high-resolution CT scanning. It is already an accepted fact that MRI provides improved imaging of the central nervous system, but, while recent articles maintain the superiority of MRI head and neck imaging, the advantages to the clinician are less clear. We present representative cases of large malignant tumors of the frontal, ethmoid, and sphenoid sinuses, in which the full extent of the disease which involved intracranial structures and the orbit was better defined on the MRI scan than it was on the high-resolution CT scan. Decisions concerning the involvement of the infratemporal fossa, the cavernous sinus, the optic chiasm, the pituitary, and the frontal lobes were more easily made when based upon magnetic resonance imaging, while the tumor involvement as evaluated by CT scanning was much less clear. Establishment of the problem regions of the head and neck--when magnetic resonance imaging is superior to high-resolution CT scanning--will assist in identification of the true need for this tool in the practice of the otolaryngologist--head and neck surgeon.