Bilateral Acute Proptosis as Initial Manifestation of Acute Myeloid Leukemia

The myeloid sarcoma is an extramedullary finding of acute myeloid leukemia (AML), and orbital leukemic tumors occur most commonly during the first decade of life. To our knowledge, we report the youngest patient with bilateral proptosis of both eyes as an initial manifestation of AML. This case highlights the need for peripheral blood smear and neuro-image work-up for acute proptosis in infancy. AML should be considered in the differential diagnosis of an orbital mass, even in the absence of typical leukemic symptoms.     

Myeloid Sarcoma in an Eyelid That Developed during Chemotherapy for Acute Myeloid Leukemia

An 80-year-old female presented with a mass in the left upper eyelid margin that had developed during chemotherapy for acute myeloid leukemia. The mass was elastic, hard, and pinkish, with a relatively smooth surface but without madarosis. The histopathological findings corresponded to a myeloid sarcoma. No blast cells were shown in the peripheral blood at the time of biopsy, and she subsequently underwent an azacitidine injection regimen. The size of the eyelid tumor decreased 3 months after the biopsy, when the course of azacitidine injections was completed. However, acute myeloid leukemia recurred, and the patient diedKey Words: Myeloid sarcoma, Acute myeloid leukemia, Chemotherapy     

Myeloid sarcoma of the orbit without systemic recurrence of disease in an adult: A clinicopathological case report

Myeloid sarcoma (MS), also known as granulocytic sarcoma (GS) or chloroma (named for the greenish hue attributed to the pigment of myeloperoxidase [MPO]), is a rare solid tumor with a predilection for the orbit. MS usually occurs in conjunction with acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), or myeloproliferative disorder (MPS) and may be the harbinger of disease. Therefore, prompt diagnosis is essential for patient survival. We present a rare case of a 61 year old with an isolated orbital MS without active leukemia.     

Granulocytic sarcoma of the orbit. A clinicopathologic study

A 9-year-old Latin American girl developed proptosis of the left eye associated with pain in the left ear of one month's duration. Ophthalmoscopic examination of the left eye disclosed retinal striae with venous tortuosity and papilledema. CT scan of the orbit showed a nonenhancing, irregular intraconal mass. CT scan of the brain demonstrated an enhancing left temporoparietal mass. Craniotomy revealed a 7 cm, extra-axial mass that was attached to the dura mater. Histologic examination, including a positive Leder stain, established the diagnosis of granulocytic sarcoma. At that time, the results of the laboratory studies, including complete cell blood count, were normal. Periodic clinical and hematologic evaluation was recommended. Twenty-five days after craniotomy, examination of the peripheral blood disclosed 56% myeloblasts and the bone marrow contained 30% blast cells. The problem in histologic differential diagnosis and a review of the literature is discussed.     

Bilateral Hypopyon as the Presenting Feature of Chronic Myeloid Leukemia

Purpose: To report bilateral hypopyon as an unusual presenting feature of chronic myeloid leukemia (CML). Design: Observational case report. Methods: A 68-year-old male presenting with bilateral hypopyon uveitis underwent hematological investigations and cytology of hypopyon. Results: Blood smear revealed increased leucocyte count with presence of abnormal cells (myelocytes, band forms, and promyelocytes) suggestive of CML. Cytopathology of hypopyon revealed predominance of lymphocytes and few plasma cells. CML was confirmed by hematological investigations. Patient was initiated on chemotherapy under the care of an oncologist and is stable. Conclusions: Elderly patients presenting with hypopyon uveitis should be investigated to rule out masquerade syndrome.     

Cutaneous Pseudolymphoma: An Unusual Eyelid Presentation

Pseudolymphoma is an inflammatory response to known or unknown stimuli that results in a lymphomatous-appearing but benign accumulation of inflammatory cells. Resemblance to lymphoma is usually most apparent histologically. Most cases are idiopathic. Approximately three-quarters of cases of cutaneous pseudolymphoma are localized with the most common site on the face (70%), chest, and upper extremities.

We would like to report an unusual eyelid presentation of cutaneous pseudolymphoma, not previously reported. It is therefore important to consider cutaneous pseudolymphoma as a differential diagnosis of eyelid lesions.     

Orbital Mass Secondary to Acute Lymphoblastic Leukaemia in a Child: A Rare Presentation

Abstract

We report the case of a 3-year-old child, who presented with lid swelling which progressed to proptosis of the left eye. He also had systemic symptoms of fatigue and weight loss. An examination revealed hepatosplenomegaly and lymph node enlargement. Investigations showed a peripheral smear with blast cells, which were also revealed through a bone marrow biopsy. A CT scan showed a mass lesion in the left orbit that had infiltrated into the surrounding tissues. He was diagnosed with acute lymphoblastic leukaemia (ALL) with left-sided orbital mass secondary to it. Haematogenous masses in the orbit are commonly due to granulocytic sarcomas, which are usually associated with acute myelogenous leukaemia (AML), not ALL, and are rare especially when they precede systemic disease.     

Bilateral Optic Nerve Involvement Combined with Unilateral Facial Palsy in a Patient with Acute Myeloid Leukaemia: A Case Report

Central nervous system (CNS) involvement, including optic nerve involvement, in a patient with acute myeloid leukaemia (AML) is an extremely rare condition. We report a case of bilateral optic nerve involvement combined with unilateral facial palsy in a patient with AML who achieved complete remission following allogenic peripheral blood stem cell transplantation as a young patient. After further evaluation, the patient was diagnosed with a recurrence of AML with CNS involvement. The presentation of multiple types of CNS involvement in AML may be suspicious evidence of AML recurrence.     

Cystic Schwannoma of Eyelid in an Indian Male: A Rare Presentation

Eyelid schwannoma (neurilemmomas) is an extremely rare, benign neoplasm. A case of Cystic Schwannoma in eyelid of a young adult is reported. A 19-year-old male presented with a firm-nodular painless lesion, 3.5 cms in diameter, located on the left lower eyelid for the last 2 years. The lesion was surgically excised. Histopathological diagnosis of cystic schwannoma was made. Immunohistochemistry revealed strong S-100 positivity indicative of Schwann cell origin. No recurrance was seen after a follow up of 1 year.     

Orbital extra-medullary granulocytic sarcoma: clinicopathologic correlation with immunohistochemical features

Orbital granulocytic sarcoma (chloroma), a rare tumor of immature myeloid cells, has nonspecific clinical and radiological features that make it a diagnostic challenge. Light microscopy and confirmation by immunostaining aid in the diagnosis. Knowledge of this entity is important because early diagnosis and prompt treatment are associated with better prognosis. We report a series of 31 biopsy-proven orbital granulocytic sarcomas with their immunohistochemical features and review the literature.     

Ocular relapse in acute myeloid leukemia (M4) with normal bone marrow

A patient with the rare occurrence of ocular relapse of acute myeloid leukemia (AML) M4 while the bone marrow was normal is reported in this paper. A 47-year-old woman with AML was treated with chemotherapy and went successfully into remission. Four months later, she presented with pain, redness, and a mass over the left eye. The ocular relapse involved the subconjunctival space and anterior chamber of the left eye and, presumably, the left lacrimal gland. There were also multiple subcutaneous nodules on both of her forearms. Incisional biopsy from the subconjunctival lesion was performed. Histopathological examination of the specimen showed diffuse blast cell infiltration. Her bone marrow was still in remission. Although exceedingly rare, ocular extramedullary relapse in AML M4 heralds bone marrow recurrence and, despite intensive chemotherapy, the prognosis is dismal. The authors have no financial interests in any of the drugs used in the management of the patient subject in this report.     

Bilateral Serous Retinal Detachment as a Presenting Sign of Acute Lymphoblastic Leukemia

We present a case of bilateral serous retinal detachment (SRD) as a presenting sign of Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph⁺ ALL). A 45-year-old woman presented with decreased vision and was found to have bilateral serous retinal detachment. Peripheral blood smears revealed leukocytosis of 53.9×10³/µL with 64.6% lymphoblasts. A bone marrow aspirate revealed the presence of lymphoblasts. Cytogenetic and molecular genetic analysis detected a reciprocal translocation between chromosome 9 and 22, t(9;22) (q34;q11). A diagnosis of Ph⁺ ALL was made. Following systemic chemotherapy, the bilateral SRD resolved completely with full recovery of vision. The sudden appearance of SRD should raise suspicion for leukemia. Prompt recognition of this disease is important for early systemic treatment and restoration of visual function.     

复合材料在烧伤后眼睑再造中的应用52例

目的:观察异体羊膜、巩膜及自体皮肤组成的复合材料在眼烧伤后眼睑再造术中的疗效及影响手术成败的因素。方法:对2001-02/2005-12我院52例(53眼)热、化学烧伤所致的眼睑挛缩、眼睑闭合不全用异体羊膜、巩膜、自体游离皮肤移植行部分眼睑上睑再造。其中酸烧伤17例(17眼),碱烧伤12例(12眼),热烧伤23例(24眼)。术中全层切开眼睑,用羊膜、巩膜、游离皮肤分别代替结膜、睑板和眼睑皮肤,置于切口中对位间断缝合以修复眼睑缺损面积。术后观察3~20mo。结果:术后40例(41眼)眼睑恢复功能,12例(12眼)眼睑再造失败。其中8例(8眼)仍有眼睑挛缩、部分眼睑闭合不全;1例(1眼)上睑成角畸形;1例(1眼)上睑下垂;2例(2眼)移植物坏死脱落。结论:用异体羊膜、巩膜、自体游离皮肤组成的复合材料行眼睑再造,手术材料来源广泛,是治疗烧伤引起眼睑挛缩的有效途径。     

儿童眼睑毛母质瘤21例病例分析

目的分析儿童毛母质瘤在眼部的临床特点。方法对我院21例病理确诊的儿童毛母质瘤患者的临床、病理及超声等表现进行回顾性研究。结果眼睑毛母质瘤占同期儿童眼睑皮下肿块(除外睑板腺囊肿)手术患者的7.9%。男女比例约为1.11.0。发生于上下睑的比例为165。术前诊断与病理检查一致的仅5例,占23.8%。组织病理观察可见典型的影细胞及嗜碱性粒细胞,伴钙化灶者18例(85.7%)。超声主要表现为低回声团块,内显示点状或斑块状高回声;或伴有低回声边缘的高回声团块。所有病例经手术切除后均未见复发。结论发生于眼睑的毛母质瘤在儿童临床并不罕见,但术前容易误诊。组织病理学及超声表现具有一定特征性表现。手术切除不易复发。     

Juvenile Xanthogranuloma Presenting with Unilateral Prominent Nodule of the Eyelid: Report of a Case and Clinicopathological Findings

Background The purpose of this study is to report and describe the clinical and histopathologic findings in a patient with juvenile xanthogranuloma presenting as a relatively large, solitary, pedunculated mass on the left upper eyelid.Case A 31-month-old Japanese girl presented with a pedunculated nodular lesion that had been developing since 4 months earlier. Otherwise, no abnormal findings were identified in either eye. In addition, no other abnormalities, including skin eruptions or nodules, were detected anywhere. The lesion was completely excised and examined histopathologically.Observations Microscopic study revealed a histiocytic proliferation accompanied by lymphocytes and occasional plasma cells and eosinophils within the dermis. A moderate number of giant cells of the foreign-body and Touton types were present. In the immunohistochemical analysis, the proliferative histiocytes and giant cells showed positive immunoreactivity for vimentin and macrophage markers and negativity for S-100 protein. The diagnosis of juvenile xanthogranuloma was made. The findings of further detailed ophthalmologic and systemic examinations and evaluations were unremarkable. Therefore, the diagnosis was made of juvenile xanthogranuloma, solitary type, occurring on the eyelid.Conclusions Although juvenile xanthogranuloma of the eyelid is uncommon and its occurrence anywhere in the eye is rare, ophthalmologists should be familiar with this entity. Juvenile xanthogranuloma should be included in the differential diagnosis of eyelid mass lesions. Jpn J Ophthalmol 2004;48:435–439 © Japanese Ophthalmological Society 2004     

A Case of Acute Myeloid Leukaemia Presenting as Subacute Intracranial Hypertension in a Young Adult

We report a case of an 18-year-old woman presenting with headache, papilloedema, and cerebrospinal fluid (CSF) pleocytosis. She was subsequently diagnosed with acute myeloid leukaemia, which is to date the only reported case manifesting as central nervous system-localised disease in an adult. The intracranial hypertension was treated successfully with chemotherapy, acetazolamide, and CSF drainage, with no permanent visual impairment. The mechanism by which haematological malignancy causes intracranial hypertension is not fully elucidated, but we hypothesise that in our case, blast infiltration interfered with CSF reabsorption at the arachnoid granulations.     

Pachydermoperiostosis: A Rare Cause of Marked Blepharoptosis and Floppy Eyelid Syndrome

Abstract

A 34-year-old African-American man was referred for eyelid swelling and ocular discomfort. He was found to have floppy hypertrophic eyelids and marked bilateral mechanical ptosis that was present since childhood. Systemic examination was significant for furrows on his forehead and scalp, coarse facial features, and enlarged hands and feet with clubbing of the fingers and toes. Radiographic imaging of the long bones demonstrated periostosis, and MRI of the head revealed a pituitary macroadenoma. Pituitary and thyroid hormone levels were normal. The patient was diagnosed with pachydermoperiostosis and a non-secreting pituitary macroadenoma. Bilateral upper lid tightening via wedge resection was followed by bilateral external levator advancement ptosis repair in a staged manner. The patient achieved symptom relief and improved lid position postoperatively.     

Unusual Presentation of Spasm of Near Reflex Mimicking Large-Angle Acute Acquired Comitant Esotropia

We report the case of an 11-year-old boy who presented with sudden esotropia, binocular diplopia, and blurred vision. The patient was neurologically normal. He had a large, constant, comitant, alternating esotropia associated with minimal accommodative spasm. Ocular motility and pupillary reactions were normal. He was diagnosed to have spasm of the near reflex presenting as acute onset of esotropia. The esotropia was persistent despite treatment and eventually resolved with prolonged cycloplegic therapy. This unusual case illustrates that spasm of the near reflex can have unique and variable presentations. Spasm of the near reflex needs to be considered in the differential diagnosis of every case of acute, acquired, comitant esotropia. This is the first case of spasm of the near reflex where persistent esotropia is reported in the absence of any neurological disorder.