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MALT lymphoma of the ocular adnexa, an indolent B-cell lymphoma, rarely affects the lacrimal gland. The case of a 73-year-old man with ptosis and edema of the left upper eyelid, due to lacrimal gland swelling, is presented. Clinical evaluation and imaging examination led to excision biopsy. The mass histopathology, presenting organized lymphoid tissue, composed mainly of small B-cells, accompanied by immunophenotypic characteristics, was compatible with MALT lymphoma. Treatment with monoclonal antibody against CD-20 achieved a successful long-term disease control (4 years). The diagnostic approach is described and the pathological features and clinical signs of this rare entity are discussed, based on recent literature. The indolent clinical course of this lymphoma, either remaining localized or disseminating to other mucosal sites, is a distinct characteristic affecting prognosis.
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PMID:Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland--a case report. 1627 31

Ptosis on its own is an infrequent initial manifestation of orbital lymphoma. Orbital lymphoma usually presents as a palpable mass with proptosis, diplopia, and conjunctival ("salmon-pink") swelling. We report here a 62-year-old female patient who presented with right eye ptosis. The initial imaging study showed an indistinct enlargement of the superior rectus-levator muscle complex. After 3-4 months, ptosis and upward gazing movement were further restricted. The imaging study revealed a definite soft-tissue mass in the superior orbit surrounding the superior rectus-levator muscle complex. A tumor biopsy through anterior orbitotomy revealed a large diffuse B-cell lymphoma. With the experience of this case, we suggest that orbital lymphoma should be included in the differential diagnosis of ptosis accompanied by impairment of levator muscle function.
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PMID:Ptosis as an initial manifestation of orbital lymphoma: a case report. 1667 2

A 55-year-old man presented with mist, ptosis, and headache. Repeated imaging studies of the brain showed wandering lesions with small hemorrhage and/or infarct-like change. Leptomeningeal enhancement was noted. Angiography revealed filling defects in dural sinuses, particularly in the left cavernous sinus. Under the diagnosis of dural sinus thrombosis accompanied with rhinitis, antibiotics and anticlotting drugs were administered. Rhinitis was improved, however, the cavernous sinus lesion remained and grew. Autopsy revealed that large B-cell lymphoma occupied the cavernous sinuses and made a mass involving sella turcica, left sphenoid bone, hypophysis. No tumor mass in the brain or tumor dissemination in the leptomeninx was observed. Intima of the brain venous system, however, was widely involved by lymphoma cells admixed with thrombi, which produced occlusion of the leptomeningeal veins and dural sinuses. Various figures of recanalization were also present. It seems that a unique type of thrombosis, i.e. tumoral thrombosis of leptomeningeal veins and dural sinuses, caused by intravascular lymphoma resulted in fatal outcome with multiple brain lesions like hemorrhagic infarct. Recanalization may partly explain transient resolutions of these multiple lesions. It may be suggested that intravascular lymphomatosis can cause marked phlebothrombosis of the brain and can mimic dural sinus thrombosis.
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PMID:[Intravascular lymphomatosis complicated with marked tumoral thrombosis of the brain venous system including dural sinuses. An autopsy case report with 5 months' follow-up and fatal outcome]. 1732 80

A 39-year-old previously healthy woman presented with a 3-year history of a painless lump in her left upper eyelid that had been increasing in size over the last 8 months. On examination, a soft, nontender mass was palpated in the medial left upper eyelid and anterior orbit. She had 2 mm of eyelid ptosis and 2 mm of inferior globe displacement. A salmon-pink mass of tissue was identified in the superomedial conjunctiva. The remaining ophthalmic examination was unremarkable. A lymphoproliferative process was suspected; however, an incisional biopsy revealed amyloid. Further workup showed no evidence of systemic amyloidosis, lymphoma, plasmacytoma, or multiple myeloma. The patient has been followed for more than 16 months with no new developments in her health. Amyloidosis has a variety of presentations in the periocular region and may be the sole manifestation of the disease process. It is not often considered in the differential diagnosis of conjunctival lesions. The classic "salmon-pink" conjunctival infitrate has been associated with lymphoproliferative disorders; however, amyloid should also be considered, as it may be clinically indistinguishable.
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PMID:Primary localized conjunctival amyloidosis simulating a lymphoproliferative disorder. 1880 72

The gold standard for the surgical treatment of prolapse of the posterior leaflet of the mitral valve (MV) for degenerative myxomatous disease has been represented by the quadrangular resection of the leaflet, according to the Carpentier technique. Since 2006 we performed a triangular resection of the prolapsing leaflet in 20 patients with myxomatous mitral regurgitation (MR). Seventeen patients (85%) underwent the triangular resection of P2; one patient (5%) had a triple scallops triangular resection (P1, P2, P3) and two (10%) a double scallops (P2, P3) resection. In this study, we report the immediate and mid-term clinical and echocardiographic results of a cohort of 20 patients, who underwent this technique. Thirty-day mortality was 0. Acute renal failure occurred in three patients (15%) and they resolved with conservative management. One patient (5%) required re-exploration for bleeding. At the mean follow-up of 13.1+/-4.2 months survival was 95%; one patient died of lymphoma during the follow-up time. All the cases were in New York Heart Association (NYHA) class I. Nineteen survivors underwent transthoracic echocardiography (TTE) (5), or transesophageal echocardiography (TEE) (13), performed by two skilled cardiologists. All patients showed no or trivial MV regurgitation. We believe that triangular resection of posterior MV leaflet (PMVL) provides excellent mid-term results providing the surgeon with a reliable and reproducible surgical option for myxomatous degenerative MV regurgitation.
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PMID:Echocardiographic assessment of mitral valve morphology and performance after triangular resection of the prolapsing posterior leaflet for degenerative myxomatous disease. 1962 42

A 35-year-old lady presented with recurrent episodes of painful right upper lid swelling and ptosis of 6 months' duration. The symptoms would subside with oral antibiotics and painkillers. She had also been undergoing treatment for one year for multiple painful osteolytic lesions in the long bones and axial skeleton for which no definite diagnosis had been made, despite several radiological and serological investigations having been performed. Computed tomographic scan showed a soft tissue lesion in the superolateral part of the right orbit with erosion of the adjacent bone. Lacrimal gland biopsy showed non-specific inflammatory changes. She was referred for full work-up to exclude lymphoma and metastasis. Magnetic resonance imaging revealed an enhancing soft tissue mass in the right anterior cranial fossa eroding the lateral orbital wall and extending into the orbit. Another mass encased the superior sagittal sinus. Bone marrow biopsy showed near total replacement of marrow cells by a round cell tumour. Immunohistochemical studies revealed that the tumour cells expressed neuron specific enolase and chromogranin. The pathological findings suggested a diagnosis of neuroendocrine tumour though neuroblastoma could not be unequivocally ruled out. (131) I-metaiodobenzylguanidine scan showed no uptake. Radiologic studies suggested multiple bony metastases. No other site of primary tumour was found so there was a strong possibility of it being a primary orbito-cranial neurogenic tumour. Neuroblastomas and neuroendocrine tumours are very rare in adults. The orbit is an unusual site for a primary of this nature. In addition, it is quite difficult to make a specific diagnosis in a less differentiated tumour.
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PMID:Rare orbitocranial tumour in an adult. 1983 15

A 35-year-old lady presented with recurrent episodes of painful right upper lid swelling and ptosis of 6 months' duration. The symptoms would subside with oral antibiotics and painkillers. She had also been undergoing treatment for 1 year for multiple painful osteolytic lesions in the long bones and axial skeleton for which no definite diagnosis had been made, despite several radiological and serological investigations having been performed. Computed tomographic scan showed a soft tissue lesion in the superolateral part of the right orbit with erosion of the adjacent bone. Lacrimal gland biopsy showed non-specific inflammatory changes. She was referred for full work-up to exclude lymphoma and metastasis. Magnetic resonance imaging revealed an enhancing soft tissue mass in the right anterior cranial fossa eroding the lateral orbital wall and extending into the orbit. Another mass encased the superior sagittal sinus. Bone marrow biopsy showed near total replacement of marrow cells by a round cell tumour. Immunohistochemical studies revealed that the tumour cells expressed neuron specific enolase and chromogranin. The pathological findings suggested a diagnosis of neuroendocrine tumour though neuroblastoma could not be unequivocally ruled out. A(131) I-metaiodobenzylguanidine scan showed no uptake. Radiologic studies suggested multiple bony metastases. No other site of primary tumour was found so there was a strong possibility of it being a primary orbito-cranial neurogenic tumour. Neuroblastomas and neuroendocrine tumours are very rare in adults. The orbit is an unusual site for a primary of this nature. In addition, it is quite difficult to make a specific diagnosis in a less differentiated tumour.
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PMID:Rare orbitocranial tumour in an adult. 2656 7

Vincristine is a vinca alkaloid used in combination with other agents in the treatment of solid tumors, lymphoma, and leukemia, as well as for idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia. A dose-limiting complication of vinca alkaloids is neurotoxicity. Vincristine is the oldest and also the most neurotoxic agent in this group. Described here is the case of a 4-year-old girl with unilateral palpebral ptosis. She has been diagnosed with precursor B-cell acute lymphoblastic leukemia. Ptosis was noted on the 45th day of therapy, and the last vincristine was administered on the 28th day of protocol 1. Vincristine-induced unilateral palpebral ptosis is a novel finding. Experience with this case suggests conservative treatment, with periodic examination, especially if ptosis is mild.
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PMID:Vincristine-induced unilateral ptosis in a child. 1993 Nov 72

This report describes a 2.8-year-old Swiss Braunvieh heifer with conjunctival changes as the lead clinical signs of malignant lymphoma. The heifer was referred to our clinic because of conjunctivitis that did not respond to treatment with antibiotics. The patient had bilateral lacrimation, severe swelling of the third eyelids and prolapse of the conjunctivae, which were erythematous and covered with haemorrhagic crusts. A clinical examination revealed enlarged prescapular and prefemoral lymph nodes. Based on cytological examination of a fine needle aspirate from a prescapular lymph node, a tentative diagnosis of malignant lymphoma was made. The heifer was euthanased and a postmortem examination carried out. The conjunctival lesions consisted of diffuse multifocal nodules of varying size. There was generalised lymphadenopathy and beige nodules were seen in several internal organs. Histological and immunohistological examinations confirmed the diagnosis of multicentric leukosis characterised by T-cell lymphoblasts, which had also infiltrated the conjunctivae. Examination of a blood sample for enzootic bovine leukosis virus yielded a negative result.
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PMID:[Conjunctival changes in a Swiss Braunvieh heifer with malignant lymphoma]. 2145 47

A 66 year old woman with end-stage renal disease and liver cirrhosis due to chronic hepatitis C virus infection was introduced to hemodialysis therapy in 2003. In 2007, hepatocellular carcinoma was identified and the patient underwent radio frequency ablation (RFA) and ethanol injection therapy (EIT) under laparotomy. A large vaginal tumor was incidentally found at gynecological examination. Histological diagnosis was diffuse large B-cell lymphoma (Stage IE). During the first course of chemotherapy, the vaginal tumor began to prolapse from the vaginal wall due to an excellent response to the chemotherapy and finally was resected. The patient received another course of chemotherapy followed by radiotherapy. The vaginal tumor was undetectable in the follow-up imaging studies. Although patients with end-stage renal disease are at increased risk for several cancers, the occurrence of malignant lymphoma following hepatocellular carcinoma is rare. Furthermore, lymphomas arising from the female genital tract are very uncommon.
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PMID:Non-Hodgkin's lymphoma of the vaginal wall in a hemodialysis patient with hepatocellular carcinoma. 2169 6


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