Cases reported "Disease Progression"

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31/53. Merkel cell carcinoma of the skin with leptomeningeal metastases.

    Merkel cell carcinoma is a rare skin tumor that is thought to arise from epithelial cells that have undergone neuroendocrine differentiation. It usually presents in older adults and has a slight male predominance. The most frequent site of occurrence is in the head and neck. It is an aggressive disease that has a high rate of local-regional and distant recurrence. Optimal treatment is controversial but generally consists of surgery and adjuvant radiotherapy. The role of chemotherapy is less defined. This report documents the first known case of Merkel cell carcinoma with perineural spread to the central nervous system with leptomeningeal dissemination. Whether this represents a more aggressive variant is unknown. Regardless, this pattern of spread is likely a rare event.
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32/53. Case study: periodic follow-up is necessary in morphea profunda to identify systemic evolution.

    The term "morphea" includes a wide spectrum of clinical entities, varying from localized plaques of only cosmetic importance to deep lesions resulting in considerable morbidity for the patient. In fact, although survival rates are no different from that of the general population, localized scleroderma may be associated with development of substantial disability, as occurs in deep morphea and in pediatric patients (disabling pansclerotic morphea of children). We report a case of morphea profunda affecting a young man with severe, rapidly progressive, widespread skin involvement and focus on the eventual systemic evolution of such cases. A 40-year-old man was admitted in 2002 for progressive subcutaneous indurations, preferentially involving the right side of the trunk. His health was altogether good, with the exception of a beginning chronic obstructive bronchopneumopathy. There was no family or personal history of dysmetabolic, cardiovascular, neoplastic, or cutaneous disease. Three years earlier, the patient had noted the appearance of two infiltrated, intensely red lesions on the right laterocervical and paraumbilical regions. These had been interpreted as subcutaneous lipomatosis on the basis of an ultrasound scan. The lesions had become progressively larger, while their surface had assumed a scleroatrophic appearance. Thereafter, other lesions had developed on his chest and lower limbs, mostly distributed on the right side of the body. Clinical examination revealed well demarcated, depressed sclerotic plaques with ivory-colored centers and erythematous borders ("lilac ring") localized on the neck, chest, and lower abdomen and limbs (Figure 1). They were bound to the deeper structures and arranged in a band-like linear distribution on the right side of the chest and abdomen where they extended horizontally for more than 10 cm in diameter. These lesions were totally asymptomatic. In addition, arborizing telangiectasias were evident on the neck and upper chest (Figure 2). Laboratory investigations provided normal range of erythrocyte sedimentation rat and C reactive protein levels and other inflammation markers. Antinuclear antibody, antidouble-strand dna, antimitochondrial, anti-extractable antigens (anti-centromere, anti-Scl-70, anti-U1RNP), and anti-borrelia burgdorferi antibodies were negative. Circulating immunocomplexes binding C1q were substantially increased. Oesophageal x-rays and lower limb electromyography were within normal limits; ventilatory function testing revealed a mild obstruction consistent with the beginning of chronic obstructive pulmonary disease. Although nailfold capillaroscopy documented nonspecific findings of connective tissue disease (mega-capillaries, segmentary dilatation and destruction), the laser-Doppler flussimetry revealed few signs of microcirculatory abnormalities, in absence of Raynaud's phenomenon. An abdominal wall ultrasonography, performed on a sclerotic plaque, documented thinning of the subcutaneous tissue, with increase of the fibrous component and lower fascia and muscle retraction. The biopsy specimen from the abdominal region included fascia and the subcutaneous tissue (previously obtained from the lower abdomen) with epidermal atrophy, a thickening and homogenization of collagen bundles in the deep dermis and hair reduction. A perivascular lympho-monocytic and plasmacellular infiltration with a dermo-epidermal distribution was present. Moreover, septal fibrosis with a perivascular lymphoplasmacellular inflammatory infiltrate was documented within the abdominal rectus muscle. The diagnosis of morphea profunda was made on the basis of clinical and histopathological findings. A therapeutic regimen based on amino benzoic potassium (Potaba; Glenwood, LLC, Glenwood, NJ), oral prednisone, and topical clobetasol was started. After several months of follow-up, the patient had obtained only moderate improvement of the clinical findings.
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33/53. Congenital sublingual teratoid cyst: a case report and literature review.

    head and neck dermoid cysts comprise less than 10% of all dermoid cysts. The sublingual location is very rare. One hypothesis for their etiology is that they arise from entrapment of epithelial debris or rests during the midline fusion of the first 2 branchial arches. They are classified as epidermoid, dermoid, and teratoid cysts. Sublingual cysts are slow-growing lesions generally seen in the second and third decades of life. They are rare in childhood. The cysts may interfere with swallowing and breathing. Rarely, they may show malignant transformation. Surgical extirpation is the treatment of choice.
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34/53. Lemierre's syndrome: the link between a simple sore throat, sore neck and pleuritic chest pain.

    An unusual case of tonsillitis which showed progression to this rare syndrome despite treatment with intravenous antibiotics. Lemierre's syndrome is a rare condition characterised by a triad of: sepsis, thrombophlebitis of the internal jugular vein along with pleuropulmonary and/or distant metastatic abscesses. diagnosis rests on a high index of suspicion and is confirmed by culture of fusobacterium spp. from blood or infected sites. Radiological investigations are established aids for confirmation of diagnosis. Treatment is with appropriate antibiotics for at least 6 weeks and surgical drainage of abscesses if required. This case has been presented to highlight the mode of presentation, diagnostic tools employed and the management of the complications that featured in this condition.
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35/53. Air bag injury as a cause of inflammatory myofibroblastic pseudotumour of the subglottic larynx progressing to myositis ossificans.

    Inflammatory myofibroblastic pseudotumour (IMFPT) is a rare condition. The clinical presentation can be indistinguishable from that of a malignant neoplasm. The most frequently affected organ is the lung; a much less common site is the larynx and subglottic involvement is particularly rare. Trauma is purportedly one potential aetiological factor, although there have been no previous reports of IMFPT occurring in the larynx secondary to external laryngeal trauma. We present a case of IMFPT of the subglottis which occurred subsequent to external trauma of the neck sustained during air bag inflation in a road traffic accident. This mechanism has not been previously reported. Furthermore, the lesion progressed uniquely to show myositis ossificans-like maturation over time. The patient was ultimately treated by means of complete local excision.
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36/53. Primary hyperparathyroidism presenting with musculoskeletal manifestations in a young patient: a case report.

    A 21 year old female patient was admitted to Tikur Anbessa Hospital with a diagnosis of primary hyperparathyroidism secondary to parathyroid adenoma. Her predominant presentation was with an anterior neck mass, diffuse osteoporosis and myopathy. The diagnosis was suggested by her clinical symptoms, physical findings, Laboratory studies and radiological survey. The tissue biopsy was also compatible with of the diagnosis. The parathyroid gland was removed surgically and the patient improved significantly. Primary hyperparathyroidism is extremely rare in young patients and presentation with myopathy is even more rare. Routine determination of serum calcium and phosphorus is recommended in patients with such presentations.
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37/53. A case of multiple cranial nerve palsy due to sphenoid sinusitis complicated by cerebral aneurysm.

    A case of sphenoid sinusitis and internal carotid aneurysm presenting with multiple cranial nerve palsy is reported. A 46-year-old man with high fever and neck rigidity developed progressive visual loss and ophthalmoplegia on both sides, and ptosis, mydriasis and facial numbness on the right side. CT scan and MRI revealed abnormal shadow of the sphenoid sinus and the cavernous sinus. Sphenoidectomy and antibiotic therapy improved left visual acuity, but the right visual disturbance remained. cerebral angiography performed 20 days after the operation, showed an aneurysm of the cavernous portion of the right internal carotid artery. This aneurysm could be called "bacterial aneurysm" due to sphenoid sinusitis and cavernous sinusitis. However, there is another possibility that the enlargement of an idiopathic and asymptomatic intracavernous aneurysm due to rapid and severe parasinusitis induced bilateral cavernous sinus syndrome with the right side dominance. After endovascular coil placement, his right visual disturbance partially recovered.
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38/53. Respiratory failure in a patient with antecedent poliomyelitis: amyotrophic lateral sclerosis or post-polio syndrome?

    We report a 69-year-old man who developed paralytic poliomyelitis in childhood and then decades later suffered from fatal respiratory failure. Six months before this event, he had progressive weight loss and shortness of breath. He had severe muscular atrophy of the entire right leg as a sequela of the paralytic poliomyelitis. He showed mild weakness of the facial muscle and tongue, dysarthria, and severe muscle atrophy from the neck to proximal upper extremities and trunk, but no obvious pyramidal signs. Electromyogram revealed neurogenic changes in the right leg, and in the paraspinal, sternocleidomastoid, and lingual muscles. There was a slight increase in central motor conduction time from the motor cortex to the lumbar anterior horn. Pulmonary function showed restrictive ventilation dysfunction, which was the eventual cause of death. Some neuropathological features were suggestive of amyotrophic lateral sclerosis (ALS), namely Bunina bodies. In patients with a history of paralytic poliomyelitis who present after a long stable period with advanced fatal respiratory failure, one may consider not only respiratory impairment from post-polio syndrome but also the onset of ALS.
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39/53. Changing clinical manifestations of a T-peripheral lymphoma: from hypereosinophilic syndrome to questionable Kimura's disease resulting in parotid mass.

    The diagnosis of low-grade lymphoproliferative disorders during a long clinical course sometimes represents a great clinical challenge. The idiopathic hypereosinophilic syndrome (HES) represents a pre-malignant state in some patients and close follow-up is necessary to detect early signs of malignant transformation. Kimura's disease (KD) mimicking HES is an immune mediated inflammatory disorder that usually involves the head and neck region, primarily affecting the salivary glands, adjacent muscle and regional lymph nodes. Clinically, it is very difficult to differentiate KD from salivary gland lymphoid malignancies. Lymphomas may uncommonly present as a parotid mass. One, herein, would like to present a 35-year-old patient with changing clinical presentation patterns over a period of more than 6 years of follow-up. The first clinical presentation of the patient was HES. The 'diagnosis' of KD was reached after 4 years of management with HES. The final manifestation was parotid gland, preauricular and cutaneous manifestations of a peripheral T-cell lymphoma. This unique clinical presentation pattern of the patient may represent the distinct pathobiological progression of a clonal neoplastic lymphoproliferative disorder.
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40/53. Acquired Chiari malformation type I following fractionated radiation therapy to the anterior skull base in a 20-month-old boy. Case report.

    The authors present the case of a 20-month-old boy who underwent fractionated radiation therapy to the paranasal sinuses and anterior skull base during treatment for nasopharyngeal parameningeal rhabdomyosarcoma. Subsequent magnetic resonance imaging demonstrated progressive development of a Chiari malformation Type I (CM-I) and partial hypoplasia of the posterior fossa. Since the tonsillar herniation was discovered, the child, now 3 years old, remains asymptomatic except for mild, intermittent neck discomfort. For the time being, his family has elected for him to undergo clinical and neuroimaging follow up. The authors believe this is the first report of a progressive acquired CM-I after cranial irradiation in the pediatric population.
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