Cases reported "Angiomatosis"

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1/22. Cystic angiomatosis of the bone appearing as intrathoracic lung masses.

    A rare case of cystic angiomatosis of the bone (CAB) manifesting as bilateral intrathoracic masses is described. In 1996, a 47-year-old woman was referred to the chest clinic at St Boniface Hospital for evaluation of chronic cough, and her chest radiograph showed large, bilateral pleural-based intrathoracic masses. Ten years before, she had been diagnosed with CAB following bone biopsies of her hip and knee. Computed tomography revealed two chest wall masses with internal septations of bone. CAB is a rare, multicentric disease characterized by involvement of the vascular and lymphatic systems, producing diffuse cystic lesions in the skeleton. Although the ribs are frequently involved, the presentation with intrathoracic masses is uncommon.
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ranking = 1
keywords = chest
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2/22. Scrape cytology of meningioangiomatosis: a report of two cases with diagnostic cytologic features.

    BACKGROUND: Meningioangiomatosis is a rare, probably hamartomatous condition characterized by nonneoplastic intracortical proliferation of meningothelial cells, capillaries and fibroblasts. The lesion may mimic a tumor both clinically and radiologically. We present two cases of the entity, including its cytologic features. CASES: A 71-year-old man presented with an eight-month history of headache, change in vision, loss of depth perception and unsteady gait. Magnetic resonance imaging (MRI) revealed an infiltrative lesion of the left occipital lobe, diffuse in nature. Cytologic smears prepared at the time of intraoperative consultation showed numerous thin-walled capillaries together with bland spindle cells. Occasional large cells with prominent nucleoli were also present. Frozen section confirmed the presence of linear, small capillaries surrounded by fibroblasts and meningothelial cells, consistent with meningioangiomatosis. The second case was a 3-year-old girl who presented with a one-day history of seizure, vomiting, fever and perioral cyanosis. MRI revealed an enhancing lesion in a temporal lobe of the cerebrum. The patient underwent excision of the lesion. Intraoperative cytology showed numerous meningothelial whorls together with neurons and occasional capillaries. Both patients were well, one after three months and the other after six. CONCLUSION: Meningioangiomatosis is a lesion characterized by linear capillaries, meningothelial cells and neurons, some exhibiting nuclear atypia. The combination can lead to an erroneous diagnosis of more common conditions, such as meningioma (with intracortical extension) and anaplastic astrocytoma. Contrary to these more sinister diagnoses, meningioangiomatosis is a completely benign, presumably hamartomatous entity.
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ranking = 2.7823977245758
keywords = headache
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3/22. A case of hemeplegic migraine with leptomeningeal angiomatosis.

    A 43 year old man with thirty years history of recurrent hemiplegic migraine, consistently occurring on one side (left sided paresthesia and weakness of less than forty five minutes followed by right sided headache) and lately increasing to once in three days was investigated. CT head and carotid angiogram showed gyral calcification with prominent medullary and subependymal veins confirming the diagnosis of leptomeningeal angiomatosis. Management and followup is discussed.
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ranking = 2.7823977245758
keywords = headache
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4/22. Cobb syndrome associated with a verrucous (angiokeratomalike) vascular malformation.

    Cobb syndrome is defined by a cutaneous vascular lesion and a corresponding spinal cord vascular malformation within a segment or two of the involved dermatome. Even though Cobb syndrome has been reported in association with various cutaneous vascular lesions, to our knowledge, only one other previous report describes verrucous changes overlying the cutaneous vascular lesion. We describe a patient with verrucous vascular malformation extending from his right chest to his right upper back in a dermatomal distribution. The patient's medical history included excision of a cavernous vascular malformation from the corresponding segment of his thoracic spinal cord 4 years prior, reversing 7 years of progressive leg weakness and neural deficits. Cobb syndrome was diagnosed based on the dermatomally distributed vascular malformation and the corresponding spinal cord vascular malformation. His skin lesions initially improved with erbium: YAG laser treatment but later recurred. This is the second reported case of Cobb syndrome associated with verrucous angiokeratomalike changes overlying the cutaneous vascular malformation. Because of the potentially severe neurologic sequelae caused by spinal cord lesions, clinicians should evaluate patients with dermatomally distributed cutaneous lesions on the trunk or extremities, including vascular malformations with verrucous features.
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ranking = 1.1681518482259
keywords = upper, chest
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5/22. Reactive angioendotheliomatosis in the setting of antiphospholipid syndrome.

    A 31-year-old man with systemic lupus erythematosus and antiphospholipid syndrome developed erythematous purpuric plaques distributed over the lower chest, abdomen and upper thighs. biopsy of lesional skin revealed intravascular proliferation of endothelial cells with associated microthrombi formation. The histological pattern was consistent with reactive angioendotheliomatosis, a rare reactive pattern seen associated with disparate medical conditions. The pathogenesis of the reactive angioendotheliomatosis in our patient was suspected to be related to his procoagulant state; thrombi formed despite a therapeutic international normalized ratio while on warfarin. His lesions began to resolve with the cessation of warfarin and commencement of subcutaneous enoxaparin, oral clopidogrel and oral aspirin. The skin biopsy findings were pivotal in influencing the change of therapy in this patient and decreasing his immunosuppression.
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ranking = 1.1681518482259
keywords = upper, chest
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6/22. A rare case of lymphangiomatosis of the craniocervical spine in conjunction with a Chiari I malformation.

    Lymphangiomatosis of the bone is rare. The axial as well as appendicular skeleton may be affected. Neurosurgical consultation may be called for several reasons: (1) lesions involving the calvarium and/or spine; (2) nondiagnostic biopsies from more accessible and less morbid locations, and (3) persistent CSF leak and/or recurrent meningitis. Thus, it is important for the neurosurgeon to be familiar with this disease entity and consider it in the differential diagnoses of multifocal lytic lesions of the axial skeleton. We present a case report of a 4-year-old girl with lymphangiomatosis of the skull base and upper cervical spine with concurrent Chiari I malformation and briefly review the literature.
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ranking = 0.83481851489261
keywords = upper
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7/22. Angiomatoid malignant fibrous histiocytoma of the eyelid.

    A 20 year old female had an angiomatoid malignant fibrous histiocytoma of her left upper eyelid extending into the orbit, frontal and temporal regions. The tumor was excised and radiotherapy was given. Nine months follow-up did not reveal any recurrence.
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ranking = 0.83481851489261
keywords = upper
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8/22. Meningioangiomatosis with meningioma: an uncommon association of a rare entity--report of a case and review of the literature.

    INTRODUCTION: Meningioangiomatosis (MA) is a rare lesion, probably of malformative origin, consisting of meningovascular proliferation and leptomeningeal calcification. patients with MA usually present with seizures or persistent headaches. Neurofibromatosis may be associated in a variable proportion of patients, while in others it may be sporadic. Surgical treatment is usually recommended, and is gratifying in most cases. Rarely, MA has been described coexisting with meningiomas, arteriovenous malformations, encephaloceles, oligodendrogliomas, meningeal haemangiopericytomas and orbital erosion. Among these, meningiomatosis with meningioma is the most frequent combination. CASE REPORT: We report a case of MA with meningioma in an 18-month-old girl, who presented with recurrent seizures. DISCUSSION: In these situations, it is extremely important for the pathologist to be aware of this entity and to distinguish it from other lesions, like cortical invasion by a meningioma, intraparenchymal meningioma and intracerebral schwannoma, which it may mimic.
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ranking = 2.7823977245758
keywords = headache
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9/22. Diffuse dermal angiomatosis of the breast.

    BACKGROUND: Diffuse dermal angiomatosis is rare and usually considered a variant of reactive angioendotheliomatosis. It generally involves the extremities of patients with severe vascular disease and other comorbidities. Two patients with breast involvement have been described; however, neither had a relevant medical history or a vaso-occlusive disorder, but both had large pendulous breasts, and 1 was positive for IgM anticardiolipin and antinuclear antibodies. OBSERVATIONS: A 53-year-old woman had a reticulated, erythematous plaque with superficial ulceration and underlying tender nodules on her left breast. She had a history of cardiovascular disease and was a heavy smoker. biopsy of the lesion showed diffuse proliferation of additional endothelial cells and small bland vessels within the papillary and upper reticular dermis. angiography showed almost complete occlusion of the subclavian artery proximally. Diffuse dermal angiomatosis was diagnosed. With isotretinoin therapy, the lesions improved. One month later, after percutaneous subclavian arterial revascularization, the lesion resolved completely. A literature review suggested that a history of heavy smoking, in addition to a history of vascular disease, may be important in the pathogenesis of diffuse dermal angiomatosis. CONCLUSIONS: Clinical acumen is crucial to diagnose diffuse angiomatosis of the breast. Appropriate treatment to alleviate hypoxia may improve the patient's condition.
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ranking = 0.83481851489261
keywords = upper
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10/22. COBB syndrome treated by staged intravascular embolisation and surgery.

    A 28-year old Malay man with evidence of an upper motor neuron cord lesion was diagnosed to have a C7 to T2 spinal arterio-venous malformation and associated cutaneous vascular lesion. He finally agreed for treatment after 5 years of progressive spastic right lower limb weakness leading to inability to mobilize. A two staged intravascular procedure was done followed by surgery with recovery of asia impairment scale grade B.
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ranking = 0.83481851489261
keywords = upper
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