Cases reported "Disease Progression"

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1/56. Borderline malignant change in recurrent mullerian papilloma of the vagina.

    Malignant change occurred in a benign, recurrent vaginal mullerian polyp. The patient, a 49 year old woman with cerebral palsy, presented with a polypoid mass in the vagina. At four years of age she had presented with a haemorrhagic polyp, and over the following years she had recurrent irregular bleeding and regrowth of the polypoidal mass, requiring a total of 10 operations to excise the polyp. Histological examination of the specimen showed typical mullerian features with tubal, endometrioid, and endocervical cell types. There were significantly abnormal nuclei, indicating low grade or borderline malignancy. review of previous biopsies showed similar mullerian features but no atypia. This is the first reported case of borderline malignant change in a previously benign recurrent mullerian papilloma of the vagina. Definitive radical surgery or radiotherapy is contraindicated in this patient and she remains under follow up.
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ranking = 1
keywords = papilloma
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2/56. Tumor growth of a choroidal malignant melanoma and aqueous flare. Report of a case.

    We report the use of the laser flare-cell meter (LFCM) in monitoring blood-ocular barrier breakdown induced by a choroidal malignant melanoma in an 88-year-old white male, who refused enucleation or radiation treatment. During a follow-up of 16 months, aqueous flare values measured with the LFCM increased from 12.8 to 26.5 photon counts/ms with continuous tumor growth from a height of 7.9 to 18 mm. Our finding of increasing flare values with tumor growth appears to confirm the observation that aqueous flare is influenced by the size of uveal malignant melanomas. Quantification of aqueous flare with the LFCM may be helpful in the follow-up of eyes with intraocular tumors.
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ranking = 0.81586760630627
keywords = choroid
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3/56. Intrahepatic bilomas associated with hepatic arteriovenous malformation.

    A 26 year-old female presented with progressive intermittent right upper quadrant pain. Hepatic arteriovenous malformation with small intrahepatic bilomas were found. She underwent hepatic artery ligation for control of her abdominal pain. Though the abdominal pain subsided after the hepatic artery ligation, the intrahepatic bilomas progressed. It is possible that the hepatic arteriovenous malformation (AVM) might reduce blood flow to the bile duct and then induce ischemia in the peribiliary capillary plexus, thus leading to bile duct necrosis and formation of bilomas, which could be further aggravated by hepatic artery ligation.
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ranking = 0.051467343795099
keywords = plexus
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4/56. Sorsby's fundus dystrophy: a case report of 24 years follow-up with electrodiagnostic tests and indocyanine green angiography.

    PURPOSE: Five families with dominantly inherited macular dystrophy were originally described by Sorsby et al. in 1949. Key features include early bilateral central visual loss secondary to either choroidal neovascularisation or central geographical atrophy and late progressive chorioretinal atrophy. We report a member of one of the original families who has been studied with a series of investigations over a long time, providing important information on differences in the phenotype and natural history of a rare genetically determined macular dystrophy. methods: The patient has been followed up for the last 24 years, from asymptomatic to full manifestation of Sorsby's fundus dystrophy. Series of fundus photographs, colour vision, dark adaptation and electrodiagnostic tests were performed. The disease was also studied with fundus fluorescein angiography and indocyanine green angiography. RESULTS: Unlike her other family members, who were reported in other studies as all having rapid loss of vision secondary to disciform macular disease, our patient has a unique clinical course in that she has a progressive bilateral central and generalised chorioretinal atrophy with a well-preserved minute central island of fovea. Nyctalopia was her early and only symptom. There was evidence of central scotoma, tritanopia and mild abnormality in dark adaptation. Rod function was affected earlier and to a larger degree than cone function. CONCLUSIONS: The overall features suggest phenotypic variability within a family in this autosomal dominant macular dystrophy. The findings from indocyanine green angiography and a consecutive series of electrodiagnostic tests in this condition support the theory of partial choroidal hypoperfusion and an interesting progressive rod-cone dystrophy as part of the pathophysiology.
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ranking = 0.32634704252251
keywords = choroid
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5/56. Central retinal vein occlusion: report of two familial cases.

    The authors report a 46-year-old father and 17-year-old son who each presented with unilateral central retinal vein occlusion (CRVO) and bilateral abnormalities of retinal vascular perfusion. The son presented with a nonperfused CRVO in the left eye, developed traction-rhegmatogenous retinal detachment treated with vitreous surgery, and developed prolonged arteriovenous filling in the retina of the fellow eye. The father presented with progressive CRVO in the right eye, developed choroido-vitreal neovascularization following laser treatment to create a chorioretinal anastomosis, underwent vitrectomy for retinal detachment and vitreous hemorrhage in that eye, and developed prolonged arm-eye and retinal arteriovenous circulation times in the fellow eye. An extensive evaluation (including hematological studies and imaging of the major vessels of the neck) failed to reveal a predisposing cause in either patient although echocardiography disclosed a mitral valve thrombus in the father. After institution of coumadin therapy, the circulatory parameters in the fellow eye of each patient improved.
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ranking = 0.16317352126125
keywords = choroid
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6/56. Grzybowski's generalized eruptive keratocanthomas: a case report.

    A 47-year old woman with Grzybowski's generalised eruptive keratoacanthomas is described. There was no history of skin disease in her family, except for an uncle's basal cell carcinoma. From 1995 she developed multiple lesions of various size, ranging from hundreds of small follicular lesions to large typical keratoacanthomas up to 5 cm in diameter, scleroderma-like facial skin and marked ectropion. Histological examination of small and large skin lesions was typical of keratoacanthoma, and no human papillomavirus was detected by polymerase chain reaction. Oral treatment with acitretin had no effect. Both cyclophosphamide and methotrexate therapy were refused by the patient despite the progressive course of the disease. Blepharoplastic surgery had some effect on eye symptoms. The etiology of this rare disease is unknown, but is probably related to some genetic defect.
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ranking = 0.2
keywords = papilloma
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7/56. Ectopic choroid plexus within a juvenile arachnoid cyst of the cerebellopontine angle: cause of cyst formation or reason of cyst growth.

    The unusual and rare case of a 6-year-old boy is reported who presented with an arachnoid cyst located in the cerebellopontine angle incorporating an ectopic piece of choroid plexus tissue. A microneurosurgical cyst wall resection was performed and the plexus tissue identified and removed. The rare occurrence of ectopic choroid plexus tissue within cysts of the CNS is discussed.
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ranking = 10.930789234765
keywords = choroid plexus, choroid, plexus
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8/56. Recurrent respiratory papillomatosis.

    Recurrent respiratory papillomatosis is characterized by the appearance of benign laryngeal squamous papillomas in childhood. lung involvement is rare. We report a case of childhood laryngeal papillomas who developed tracheobronchial papillomas and a nodule in the lung after a period of 21 years. Frequent sampling of pulmonary lesions to detect malignant transformation is suggested as prognosis of lung lesions are worse in comparison to laryngeal papillomas.
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ranking = 1.8
keywords = papilloma
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9/56. Brachial plexopathy associated with diffuse edematous scleroderma.

    peripheral nervous system (PNS) involvement is rare in systemic sclerosis (SSc), usually restricted to peripheral nerve entrapment. To our knowledge, only one case of scleroderma with brachial plexus involvement has been reported previously. We report here on a 61-year-old woman with past history of limited cutaneous SSc who developed motor deficiency in the left arm concomitant with diffuse edematous scleroderma without evidence for trauma or compression of the brachial plexus. After six months intravenous pulse cyclophosphamide therapy, dramatic improvement of skin and neurological involvement was observed.
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ranking = 0.1029346875902
keywords = plexus
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10/56. Rapid progression of primary vaginal squamous cell carcinoma in a young hiv-infected woman.

    BACKGROUND: The association of human immunodeficiency virus (hiv) infection with rapid progression of cervical and anal squamous cell carcinoma has been clearly established by several studies. Human papilloma virus (HPV) infection of the anogenital tract is believed to be the causative agent of cervical, anal, vaginal, and vulvar squamous cell carcinoma. While a myriad of reports exist in the literature pertaining to the rapid progression of cervical and anal carcinoma in hiv-infected patients, no association of hiv infection and vaginal carcinoma has been reported. We present an unusual case of a young woman infected with hiv who was diagnosed with advanced vaginal carcinoma and succumbed to her disease shortly thereafter despite aggressive treatment. CASE: A 40-year-old woman with a 2-year history of hiv infection presented with Stage IVA squamous cell carcinoma of the vagina and a large vesicovaginal fistula from the tumor eroding through the posterior bladder wall. Computed tomography (CT) of the abdomen and pelvis revealed a large tumor replacing the vagina with mild hydronephrosis and diffuse pelvic and inguinal lymphadenopathy. She underwent urinary diversion with a transverse colon conduit followed by pelvic radiation with weekly cisplatin chemosensitization. A repeat CT scan of the abdomen and pelvis upon completion of her treatment revealed progression of disease with multiple liver metastases and gastrohepatic ligament adenopathy. She subsequently died of advanced metastatic vaginal carcinoma 2 months after completion of treatment. CONCLUSION: Due to the rarity of primary vaginal carcinoma, the clinical behavior of this neoplasm in the hiv-infected patient is poorly understood. Our case indicates that, although vaginal carcinoma is a disease of the elderly, young women infected with hiv and HPV are predisposed not only to develop cervical or anal carcinoma but also may be at increased risk for vaginal carcinoma with more aggressive and less responsive disease. Furthermore, although vaginal carcinoma is usually a slow-growing neoplasm, this case illustrates the aggressive behavior of such a tumor when associated with hiv infection.
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ranking = 0.2
keywords = papilloma
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