Juvenile nasopharyngeal angiofibroma. Awareness in anesthesia of the juvenile nasopharyngeal angiofibroma.
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Adolescent↗
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Juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis: an association?
Juvenile nasopharyngeal angiofibroma is a benign neoplasm affecting the nasopharynx of male adolescents. Two patients treated at Temple University Hospital for this condition were also diagnosed with familial adenomatous polyposis. Familial adenomatous polyposis results from the inheritance of a mutated adenomatous polyposis coli gene in an autosomal dominant pattern. The development of colorectal carcinoma in middle age is seen almost invariably in familial adenomatous polyposis, if a prophylactic colectomy is not performed. To identify a possible association between juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis, chart reviews and patient interviews were carried out for all patients treated for juvenile nasopharyngeal angiofibroma at Temple University Hospital between 1985 and 1993. Single-strand conformational polymorphism was performed to detect the presence of certain adenomatous polyposis coli gene mutations within the germline DNA of those juvenile nasopharyngeal angiofibroma patients not previously found to have familial adenomatous polyposis. Although no more patients with both juvenile nasopharyngeal angiofibroma and familial adenomatous polyposis were found by these methods, the two patients with both disorders previously identified constitute 22% of our juvenile nasopharyngeal angiofibroma series. The implications of these findings are discussed.
Adenomatous Polyposis Coli↗
The size of small polydisperse circular DNA (spcDNA) in angiofibroma-derived cell cultures from patients with tuberous sclerosis (TSC) differs from that in fibroblasts.
Cell cultures were derived from angiofibromas of three patients with tuberous sclerosis (TSC), from the unaffected skin of these patients, and from the skin of five healthy donors. The length distributions of the small polydisperse circular DNA (spcDNA) fraction of these cell cultures were then analyzed. Nearly half the spcDNA molecules from the angiofibroma cultures were longer than 0.4 micron, whereas only about 7% exceeded this threshold in the spcDNA preparations from the skin fibroblast cultures. The percentage of the larger size class of spcDNA showed an increase at higher numbers of in vitro passages in all three types of cultures, but this effect was much more conspicuous in the angiofibroma-derived cultures than in those from the skin fibroblasts. An age-dependent increase in the overall amount of spcDNA was only seen in the angiofibroma-derived cultures. Our earlier finding of elevated amounts of spcDNA in angiofibroma cultures was confirmed in cultures from an additional TSC patient.
Adult↗
Nasopharyngeal angiofibroma in patients with familial adenomatous polyposis.
Four patients with nasopharyngeal angiofibroma and familial adenomatous polyposis are reported here. Nasopharyngeal angiofibroma was 25 times more frequent in our patient population with familial adenomatous polyposis than in an age-matched hospital population. The association of these two rare conditions suggests that nasopharyngeal angiofibroma is an extracolonic manifestation of adenomatous polyposis. In addition, somatic mutation of the adenomatous polyposis coli gene, which causes adenomatous polyposis when mutated in the germline, could play a role in the pathogenesis of sporadic nasopharyngeal angiofibroma.
Adenomatous Polyposis Coli↗
Sex hormones in juvenile nasopharyngeal angiofibroma tissue.
Five cases of juvenile nasopharyngeal angiofibroma were studied in terms of the presence of progesterone, estradiol, testosterone, and dihydrotestosterone in the juvenile nasopharyngeal angiofibroma tissue using the peroxidase-antiperoxidase method. Progesterone and estradiol were positive in all cases. Testosterone was positive in 2 of the 5 patients. Dihydrotestosterone was positive in 3 of the 5 patients. Hormone in the juvenile nasopharyngeal angiofibroma tissue seems to change by the activity of nasopharyngeal angiofibroma.
Adolescent↗
[Nasal angiofibroma in an adult].
We have studied a nasal Angiofibroma in a 71-years-old male, who had not undergone any male-hormonal-therapy. The age of occurrence and the clinical course of this case made the tumor very unlikely to be a silent puberal nasal angiofibroma. After morphologic exam and exclusion of other bleeding entities of the nasal cavity we arrived at the diagnosis of nasal angiofibroma. We believe that this tumor might be arisen "ex novo" and that the nasal angiofibroma of elderly people might be a new entity.
Aged↗
Sarcomatous transformation of nasopharyngeal angiofibroma.
A case of fibrosarcoma arising in a recurrent nasopharyngeal angiofibroma 18 years after radiation therapy is described. A review of the medical literature revealed two other documented cases of sarcomatous transformation of angiofibroma, and in both, the angiofibromas had also been irradiated before the sarcomatous transformation. These occurrences should caution against the indiscriminate application of radiation therapy in nasopharyngeal angiofibromas.
Histiocytoma, Benign Fibrous↗
[Electron microscopic examination on cytogenesis of juvenile nasopharyngeal angiofibromas (author's transl)].
After the electron miscroscopic examination of tissue samples of juvenile nasopharyngeal angiofibromas obtained from 9 male patients ranging in their age from 7-24 years the problems of cytogenesis and classification are discussed. Besides it is tried to correlate particular morphological findings to certain clinical phenomenons. The vascular component of juvenile nasopharyngeal angiofibromas shows a clear proliferation of the vascular wall cells. Particularly, proliferating pericytes, cells withous peculiar characteristics ("undifferentiated" cells) and cells in various stages of differentiation are to be emphasized. Obviously, vascular wall cells emigrate into the surrounding tissue and transform themselves into small fibroblasts. The second component of juvenil nasopharyngeal angiofibromas is represented by stromal fibroblasts with several cytological variations. Only activated "classical" fibroblasts and fibroblasts with histocyte-like features reveal the nuclear pattern unique for these growths which is characterized by the combination of protrusions of nuclear membrane with formation of nuclear "blebs" and of dense intranuclear granules. Cells with these nuclear characteristics were considered as preexisting fibroblasts. Thus juvenile nasopharyngeal angiofibromas are formed by the proliferation of two tissue components, namely by the proliferation of vascular wall cells and stromal fibroblasts, and can be conceived as reactive hyperplasias. The swelling body-like and organoid appearance, cytological pecularities, characteristic topographic relations (localization and supplying vessesl) and the sex-dependent occurrence speak for a tumor-like hyperplasia of a rudimentary organ unknown till now.
Adolescent↗
Estrogen receptors in nasopharyngeal angiofibromas.
Nasopharyngeal angiofibromas, which occur exclusively in pubescent males, are frequently treated with estrogens in an effort to decrease their size and vascularity. It has been presumed by some that estrogen has a direct effect on the tumor itself. Others have felt that hormone acts by affecting the pituitary-gonadal axis. In this study, six male patients with nasopharyngeal angiofibromas had their tumors analyzed for estrogen receptors and none were found. Despite this fact, two of these patients treated with estrogens showed clinical evidence of a decrease in tumor size and vascularity. This response occurring in the absence of estrogen receptors suggests that the action of estrogen on nasopharyngeal angiofibromas is indirect. Alternatively these tumors may be testosterone dependent and the administration of estrogen suppresses the normal male testosterone levels, with a resultant reduction in tumor size and vascularity. Twenty-four patients with nasopharyngeal angiofibroma have been treated at the University of Virginia in the past 22 years. The average blood loss at operation was 1900 cc. When estrogens were administered preoperatively in 3 patients, the average blood loss was 1135 cc. Those patients who received no estrogens averaged 2000 cc blood loss. These figures do not reflect the size or extent of the disease, both of which greatly influence blood loss.
Adolescent↗
Malignant transformation of a nasopharyngeal angiofibroma.
The malignant transformation of a nasopharyngeal angiofibroma is a rare occurrence. This report describes the development of a fibrosarcoma in a patient over a 20-year period after four surgical procedures and two courses of radiation therapy to control a nasopharyngeal angiofibroma. A review of the literature revealed similar descriptions of this problem. Radiation likely plays a major role in causing the malignant change. An initial complete surgical excision can prevent recurrence of a nasopharyngeal angiofibroma as well as sarcomatous transformation. With the availability of surgical procedures that can completely and safely resect almost all nasopharyngeal angiofibromas, radiation therapy can be avoided.
Adolescent↗
[Nasopharyngeal angiofibroma. Histochemical observations and hormone therapy (author's transl)].
There were carried out substrate- and enzymhistochemical reactions of the typical nasopharyngeal angiofibroma in 6 cases. In the environs of the vessels there were mainly neutral mucopolysaccharides, in the left intercellular substance and in the fibroblasts and most cells acid carbohydrates were found. The protein reactions pointed out a conspicouos strong contents of alkaline proteins in fibroblasts, mast cells and vessel environs. In the mast cells and fibroblasts tryptophan was found plentifully, in fibroblasts and vessel environs a lot of tyrosin was provable. The alakline phosphatase was traceable in fibroblasts and mast cells with great intensity. Strong reactivities of the acid phosphatases in all of the cells of the tissue refer to intensive lysosomal activities and considerable secretion transactions. The reaction of LDH as a representative of energy metabolism settles down in all of the cells and vessel environs in a strong way. The histochemical examination of a preparation of the naso-pharyngeal angiofibroma after hormone therapy showed a falling out of all the enzyme activities. The reduction of the acid phosphatases seems to be of special importance. Substrathistochemically there weren't any clear modifications concerning quality and quantity. A comparatively examined separation of an ordinary fibroma of the nasopharynx indicated similar enzymhistochemical pictures disregard the intensive reaction of the unspecific esterase, which doesn't appear concerning the nasopharyngeal angiofibroma. The carbohydrate reactions demonstrated some differences to the typical nasopharyngeal angiofibroma.
Acid Phosphatase↗
Juvenile oral angiofibroma in a female.
A case of oral angiofibroma in a 14-year-old girl is presented. Unlike in earlier reports, the patient was confirmed as female by gynecological examination and other specific examinations. The possibility of the origin of the angiofibroma from the Meckel's cartilage is postulated. Any exuberant, sessile growth with a tendency toward spontaneous bleeding should be viewed with suspicion in terms of angiofibroma. Lesions such as pyogenic granulomas, hemangiomas, myxomas, and fibromyxomas should be kept in mind while making a diagnosis of angiofibroma. A biopsy examination as a routine office procedure should not be done on benign-looking masses with tendencies to bleeding.
Adolescent↗
[Retrospective clinicopathological study of juvenile nasopharyngeal angiofibroma in a 20-year case load].
From the files of our institution 7 cases originally diagnosed as juvenile nasopharyngeal angiofibroma have been reviewed. Only 3 cases proved to be juvenile nasopharyngeal angiofibroma on revision, in one case inflammatory polyp, in two cases pyogenic granuloma and in one case richly vascular granulation tissue was the correct diagnosis. The clinical and histopathological aspects of the differential diagnosis of juvenile nasopharyngeal angiofibroma are discussed. All patients with true juvenile nasopharyngeal angiofibroma were males. The diagnostic usefulness of the recognition of morphological features suggesting erectile tissue are stressed.
Adolescent↗
Radiation therapy as an alternative to surgery in the management of intracranial juvenile nasopharyngeal angiofibroma.
Juvenile nasopharyngeal angiofibroma is a benign, vascular tumor which typically presents in adolescent males. Although surgical resection is usually recommended for the management of this tumor, external beam radiation therapy has also been advocated in the literature. We report three cases of large juvenile nasopharyngeal angiofibromas with extensive intracranial extension primarily managed with external beam radiation therapy. Although there was not complete resolution of the tumors, there was significant alleviation of symptomatology with no serious side effects from the radiation therapy. Based on these cases, we feel that external beam radiation therapy in the management of extensive juvenile nasopharyngeal angiofibromas with intracranial extension is warranted in certain select cases.
Adolescent↗
Surgical experience with nasopharyngeal angiofibroma.
Recent reports have suggested that radiotherapy may be preferable to other forms of treatment of juvenile nasopharyngeal angiofibroma. There are, however, potentially serious short- and long-term complications associated with the use of radiotherapy in the head and neck. For the past three years, ten consecutive patients with juvenile nasopharyngeal angiofibroma have been treated at The Cleveland Clinic Foundation with an approach that permits accurate removal with minimal complications. With this method of treatment, intraoperative blood loss, the necessity for blood transfusion, and length of hospital stay have been greatly decreased. There have been no substantive complications and no recurrences to date. If further experience with this approach to management in a larger series of patients has the same results, it would seem that radiotherapy should be relegated to a secondary position in the treatment of juvenile nasopharyngeal angiofibroma, except for cases wherein intracranial extension would prevent total excision.
Adolescent↗
Juvenile nasopharyngeal angiofibroma tumor models. Failure of androgens to stimulate growth in nude mice and in vitro.
Juvenile nasopharyngeal angiofibroma is a tumor with a predilection for adolescent boys. It has been shown to contain cytosolic androgen receptors and to regress with estrogen therapy; however, the results have not been consistent. Extensive investigation has been unable to settle this issue in patients owing, in part, to the rarity of these tumors. We have attempted to establish a tumor model for juvenile nasopharyngeal angiofibroma by transplanting the tumor into the subdermal space of athymic mice and also by culturing it in vitro, to study the effect of hormonal manipulation. The tumor did survive in male and female athymic mice but has failed to grow. Androgen treatment of the mice of either sex did not alter its survival or growth behavior. The in vitro tissue culture grew fibroblastoid cells that were not stimulated by androgen supplementation. This study suggests that factors other than androgens are at least complementary, if not essential, in promoting the growth of juvenile nasopharyngeal angiofibroma in tumor models, and that androgens are not, in and of themselves, sufficient growth stimuli.
Animals↗
Hormonal receptor determination in juvenile nasopharyngeal angiofibromas.
Juvenile nasopharyngeal angiofibromas (JNA) are rare. They have been frequently treated with estrogens, either solely or as an adjuvant therapy prior to surgery or irradiation. Clinical trials have proveded no evidence to explain the objective respose to estrogens observed in some tumors. Since the mechanism of steroid hormone action is mediated via specific receptors, we analyzed 8 JNA for tumor cytosol estrogen receptors. None were positive for estrogen receptors. Additionally, all were also negative for progesterone receptors. Nasopharyngeal angiofibromas occur predominantly in adolescent boys at a time when there is a gradual change in androgen availability. Therefore, three latter angiofibromas were also analyzed for the presence of cytosol androgen receptor. Specific testosterone and dihydrotestosterone binding components in the tumor cytosol were detected. This observation raises for the first time the possibility that JNA may be an androgen-dependent tumor. Estrogen may act as an antiandrogen on these tumors, an action similar to that on prostate cancer.
Adolescent↗
Computerized axial-tomography in the preoperative evaluation of an angiofibroma.
Computerized axial tomography (CAT) of the head provides the otolaryngologist with an effective diagnostic tool. This technique is particularly useful when used in the preoperative evaluation of head and neck pathology and may be used to define the extent of disease and to direct the surgical approach. The case of a 16-year-old male with an extensive angiofibroma involving the paranasal sinuses and nasopharynx is presented as an example of CAT in preoperative planning of the surgical procedure. Computerized tomography was used initially to define the tumor margins. Serial CAT scans were also obtained at intervals during the patient's preoperative estrogen therapy. Evidence was obtained which suggests CAT may be used to monitor the effects of preoperative hormone management of angiofibromas and in the definition of the optimal time for surgery. In the case presented the final CAT scan provided a detailed and accurate description of the tumor margins which were confirmed at surgery. Our experience with a case of an extensive nasopharyngeal angiofibroma demonstrates the CAT scan to be a useful objective diagnostic method for preoperative planning.
Adolescent↗