1/49. Recurrent juvenile-onset laryngotracheal papillomatosis with transformation to squamous cell carcinoma of the lung.A 47-year-old female patient with recurrent juvenile-onset laryngotracheal papillomatosis for 27 years had multiple bilateral pulmonary lesions, the largest of which was a well-differentiated squamous cell carcinoma. This case is unique because the malignant transformation occurred in a nonirradiated, nonsmoking patient.- - - - - - - - - - ranking = 1keywords = papilloma (Clic here for more details about this article) |
2/49. Malignant transformation in non-irradiated juvenile laryngeal papillomatosis.A spontaneous cancer transformation of non-irradiated juvenile papillomas has been observed in a very small number of cases. We report on six more patients with juvenile laryngeal papillomatosis developing into a squamous-cell carcinoma. Three patients had childhood papillomatosis and three patients adult onsets of their papillomas. The average duration between onset of papillomatosis and cancer diagnosis was 33 years, while the average age at time of diagnosis of a laryngeal cancer was 50 years. All patients were cigarette smokers, but none of them had received prior irradiation. The typical histological picture of tumors showed an infiltrating keratinizing squamous carcinoma besides remaining benign papillomas within the larynx. Our study again illustrates the risk of malignant transformation of juvenile papillomatosis in long-lasting cases. Therefore, regular extensive biopsies and careful histopathological examination are required. The role of smoking as a co-factor in the development of carcinoma ex-papilloma is still not clear.- - - - - - - - - - ranking = 2.4keywords = papilloma (Clic here for more details about this article) |
3/49. Malignant evolution of choroid plexus papilloma.choroid plexus tumors are rare CNS neoplasms. The distinction between choroid plexus papilloma (CPP) and choroid plexus carcinoma (CPC) is made on the basis of clinical and histological criteria. Malignant evolution of CPP may occur, and the presence of mitotic figures in CPP may predict the likelihood of recurrence or malignant evolution. Close surveillance is mandated for these patients. We report on two such cases of CPP that transformed to CPC at the time of recurrence.- - - - - - - - - - ranking = 4.8070943460483keywords = choroid plexus papilloma, papilloma, choroid plexus, plexus papilloma, choroid, plexus (Clic here for more details about this article) |
4/49. Malignant melanoma arising in a choroidal magnacellular nevus (melanocytoma).A case is presented of a 55-year-old Caucasian male whose right eye was enucleated for a mixed spindle-A and spindle-B malignant melanoma in 1967. The ophthalmoscopic picture, fluorescein angiography, and overlying peculiar orange pigmentation were suggestive of a malignant lesion. Study of serial sections of the entire lesion made possible the histologic reconstruction of the lesion, clinicopathologic correlation of the yellow-orange pigment, and the location of the nevus and mixed spindle-cell melanoma components.- - - - - - - - - - ranking = 0.078426403221154keywords = choroid (Clic here for more details about this article) |
5/49. Giant congenital pigmented naevus with unusual presentation and early malignant transformation in a Nigerian infant: case report and review of the literature.A six month-old female infant with a giant congenital pigmented naevus is reported because of the rarity and unusual presentation and early malignant transformation. It was the first case to be documented in our centre. There was an extensive giant, darkly pigmented naevus of the "bathing trunk" distribution noted at birth, associated with multiple darkly pigmented smaller naevi scattered over the trunk and limbs. Bv the third month of life large pigmented lesion began to demonstrate small papillomatous projections studding the entire surface and at age six months nodular and warty excrescences appeared including a large pedunculated spherical mass attached to the anal verge. The labia majora became anatomically disfigured by these nodular, warty lesions of various sizes associated with the large pigmented hairy naevus. Histological examination of the excised large perianal and vulvar nodules showed the typical picture of an intradermal naevus consistent with giant congenital pigmented (melanocytic) naevus. The deeper portions of the lesion showed features of malignant transformation. The rapidity of evolution of the lesions including histologic features of early malignant transformation and the gross disfiguring of the genitalia and perineum are uncommon presentation of this melanocytic naevus. In view of the early malignant transformation immediate surgical treatment was indicated. Unfortunately the patient was lost to follow-up. The case report is complimented with a review of the literature.- - - - - - - - - - ranking = 0.2keywords = papilloma (Clic here for more details about this article) |
6/49. Tissue-specific expression of SV40 in tumors associated with the li-fraumeni syndrome.Inactivation of wild-type p53 tumor suppressor function is the primary mechanism of tumor initiation in li-fraumeni syndrome (LFS) individuals with germline p53 mutations. Tumors derived from LFS patients frequently retain the normal p53 allele, suggesting that alternative mechanisms in addition to gene deletion must be involved in inactivating wild-type p53 protein. dna tumor viruses, such as SV40, target p53 for inactivation through the action of viral oncoproteins. We studied the probands from two unrelated LFS families, each of whom presented with multiple malignant neoplasms. Patient 1 developed an embryonal rhabdomyosarcoma (RMS) and a choroid plexus carcinoma (CPC), while patient 2 developed a CPC and subsequently presented with both an osteosarcoma (OS) and renal cell carcinoma (RCC). We utilized DNA sequence analysis and immunohistochemistry to determine p53 gene status in the germline and tumors, as well as evidence for SV40 T-antigen oncoprotein expression. Each patient harbored a heterozygous germline p53 mutation at codons 175 and 273, respectively. In patient 1, the normal p53 gene was lost while the mutant p53 allele was reduced to homozygosity in the RMS. Both normal and mutant genes were maintained in the CPC. In patient 2, normal and mutant p53 alleles were retained in both the CPC and RCC. Both specific PCR and immunostaining detected SV40 T-antigen in both CPCs and the RCC. In addition to chromosomal alterations, epigenetic mechanisms may disrupt p53 function during tumorigenesis. In two LFS patients, we found SV40 DNA sequences and viral T-antigen expression that could account for inactivation of the normal p53 protein. Inactivation of p53 or other tumor suppressors by viral proteins may contribute to tumor formation in specific tissues of genetically susceptible individuals.- - - - - - - - - - ranking = 0.20343656971585keywords = choroid plexus, choroid, plexus (Clic here for more details about this article) |
7/49. Inverted papilloma invading the orbit.Inverted papilloma is an unusual entity for many ophthalmologists. We report a case of inverted papilloma of the nasal cavity and sinuses, invading the orbit in a 33-year-old man. The patient presented with a right medial canthal mass, proptosis, diminution of vision, epiphora and nasal obstruction. In the past, he underwent multiple procedures for surgical removal of the tumor with frequent recurrences. A computed tomographic scan revealed a mass filling both the nasal cavities and sinuses, destroying adjacent bones and invading the medial aspect of the orbit, leading to proptosis. Excision of the tumor was performed by a lateral rhinotomy approach. The tumor proved to be an inverted papilloma with focal transformation to transitional cell carcinoma. Post-operative CT scan revealed residual tumor, which responded to radiotherapy, with no recurrence over six months. Histopathologically, these tumors are benign, but locally invasive. The tumors that invade the orbit are usually locally aggressive, highly malignant and recur frequently. Radical excision of the tumor is technically difficult and often incomplete, so radiotherapy should be considered as an adjunct to surgery.- - - - - - - - - - ranking = 1.4keywords = papilloma (Clic here for more details about this article) |
8/49. epidermodysplasia verruciformis: 14 members of a pedigree with an intriguing squamous cell carcinoma transformation.A 38-year-old farmer was apparently well until 5 years of age. At this age, the patient's mother found mildly itchy, raised eruptions over the scalp during combing of the scalp hair. Since then, the eruptions have progressed insidiously to involve the face and other parts of the body, with a preponderance over the face, upper back, and chest, including the palms. The soles, oral cavity, and genital tract were unaffected. The lesions were persistent in nature and showed no remission or exacerbation. Mild to moderate pruritus/discomfort was experienced following sunlight exposure. A prominent, raised eruption appeared on the right side of the forehead 9 years ago, 25 years after the initial eruptions, which in due course ulcerated. It was progressive in nature and acquired a large size. Two years later, it was diagnosed as squamous cell carcinoma, for which liquid nitrogen cryosurgery was performed. There was a recurrence of the lesion at the site of surgery, which was excised 4 months later. Subsequently, there was a sudden flare up at the same site. It was badly infected with maggot infestation. The relentless course of the disease was evident by the appearance of two similar lesions, one on the right half of the nose and the other on the left preauricular region. A short while ago, fatigue and loss of weight were also recorded. Bladder and bowel functions were normal and there was no loss of appetite, hoarseness of voice, or breathlessness. Four of the patient's six children (three sons and one daughter) were reported to have similar lesions all over the body. In addition, nine other relatives were also affected. Accordingly, a total of 14 (12 males and two females) family members were found to be affected from amongst 41 individuals (26 males and 15 females) spread across several generations (Fig. 1). There was a second-degree consanguinity of marriage, with the patient's grandmother and wife's father being brother and sister. skin surface examination was marked by multiple, discrete, flat-topped, scaly, brownish-black papules of diverse morphology, from hypopigmented macules to verrucous lesions, with a few coalescing to form plaques. The scales were brown, dry, and adherent (Fig. 2a). The lesions were conspicuous by their presence over the face, neck, and front and back of the chest. The extremities were also affected. Nevertheless, the soles and genitalia were spared. The oral mucosae, hair, and nails were normal. Koebner's phenomenon was explicit, whereas Auspitz's sign was not demonstrable. In addition, a perceptible nodulo-ulcerative lesion (size, 3 in x 2 in) with indurated, raised, averted margins was encountered on the right side of the forehead. The ulcer was tender and had a fetid discharge. Necrotic slough was prominent over its base. Similar lesions were located on the left preauricular region and right half of the nose. hematoxylin and eosin-stained sections prepared from biopsies taken from representative lesions of epidermodysplasia verruciformis and squamous cell carcinoma revealed their diagnostic features. epidermodysplasia verruciformis showed features of hyperkeratosis and acanthosis with hardly any papillomatosis. Marginal elongation of the rete ridges was present. Extensive vacuolization was a remarkable feature, and was largely confined to the upper stratum Malpighian and the granular cell layer. Some of the vacuolated cells were fairly large; their nuclei were located in the center and had variable pyknosis. The granular cell layer was uniformly thickened, whereas the stratum corneum had a loosely felted (basket-weave) appearance. The dermis was apparently normal (Fig. 2b,c). The other microsection(s) from squamous cell carcinoma were conspicuous by the presence of hyperkeratosis, acanthosis, and irregular masses of epidermal cells, proliferating downwards and invading the dermis. Varying proportions of differentiated squamous cells formed their bulk. These cells were atypical, characterized by variations in size and shape, hyperplasia and hyperchromasia of the nuclei, absence of prickles, chas, characterization of individual cells, and the presence of both mitotic and atypical mitotic figures (Fig. 3b).- - - - - - - - - - ranking = 0.2keywords = papilloma (Clic here for more details about this article) |
9/49. Pulmonary involvement in a case of juvenile-onset recurrent respiratory papillomatosis.Juvenile-onset recurrent respiratory papillomatosis is primarily a disease of children and adolescents. Although most patients experience spontaneous regression at a later age, some patients continue to be affected throughout their lives. We report the case of a 35-year-old woman with a 33-year history of juvenile-onset recurrent respiratory papillomatosis who developed pulmonary dissemination with malignant transformation. Malignant transformation is the most feared sequela of pulmonary dissemination, and it should be addressed aggressively. If treated promptly, the patient can enjoy prolonged survival. Computed tomography is superior to plain radiography for detecting and evaluating the extent of disease.- - - - - - - - - - ranking = 1.2keywords = papilloma (Clic here for more details about this article) |
10/49. Remote intrapulmonary spread of recurrent respiratory papillomatosis with malignant transformation.Recurrent respiratory papillomatosis is the most common neoplasm of the larynx in childhood. Extension into lung parenchyma occurs in less than 1% of patients and has a low risk of malignant transformation. Treatment options for intrapulmonary spread have shown limited success. We describe a case of recurrent respiratory papillomatosis with extensive parenchymal involvement and adenosquamous carcinoma in a 14-year-old girl.- - - - - - - - - - ranking = 1.2keywords = papilloma (Clic here for more details about this article) |
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