Development of a core outcome set for cutaneous squamous cell carcinoma trials: identification of core domains and outcomes.

BACKGROUND: The lack of uniformity in the outcomes reported in clinical studies of the treatment of cutaneous squamous cell carcinoma (cSCC) complicates efforts to compare treatment effectiveness across trials. OBJECTIVES: To develop a core outcome set (COS), a minimum set of agreed-upon outcomes to be measured in all clinical trials of a given disease or outcome, for the treatment of cSCC. METHODS: One hundred and nine outcomes were identified via a systematic literature review and interviews with 28 stakeholders. After consolidation of this long list, 55 candidate outcomes were rated by 19 physician and 10 patient stakeholders, in two rounds of Delphi exercises. Outcomes scored 'critically important' (score of 7, 8 or 9) by ≥ 70% of patients and ≥ 70% of physicians were provisionally included. At the consensus meeting, after discussion and voting of 44 international experts and patients, the provisional list was reduced to a final core set, for which consensus was achieved among all meeting participants. RESULTS: A core set of seven outcomes was finalized at the consensus meeting: (i) serious or persistent adverse events, (ii) patient-reported quality of life, (iii) complete response, (iv) partial response, (v) recurrence-free survival, (vi) progression-free survival and (vii) disease-specific survival. CONCLUSIONS: In order to increase the comparability of results across trials and to reduce selective reporting bias, cSCC researchers should consider reporting these core outcomes. Further work needs to be performed to identify the measures that should be reported for each of these outcomes.

Fibrous histiocytoma of the eyelid obliterating the punctum and the canaliculus.

A 17-year-old boy with a growth in the medial right lower eyelid obliterating the lower punctum and canaliculus underwent incisional biopsy, which revealed fibrous histiocytoma. The remaining tumor was excised and the eyelid was reconstructed. No recurrence was noted after 1 year of follow-up.

Orbital emphysema following remote skull trauma.

In an unusual case of orbital emphysema following nose blowing, a reliable patient history and examination demonstrated no direct trauma to the orbit. Blunt posterior skull trauma was sustained several hours before the development of the orbital emphysema. A "seismic" transmittal of force to the orbital walls is postulated.

Granulocytic sarcoma is associated with the 8;21 translocation in acute myeloid leukemia.

PURPOSE: Since the only three cases of granulocytic sarcoma among patients with acute myeloid leukemia (AML) seen at our institution during the last 12 years were each associated with the 8;21 translocation [t(8;21)], we sought to determine if this association is specific and more frequent than previously recognized. PATIENTS AND METHODS: We report three patients with AML and t(8;21) who developed granulocytic sarcomas, and review the world literature. RESULTS: Between 1980 and 1992, 53 cases of AML French-American-British (FAB) M2 were identified at our institution. Eight (15%) patients had t(8;21). Three of these eight patients (38%) developed granulocytic sarcoma. All three of our patients received conventional intensive antileukemic chemotherapy yet had short relapse-free survival durations. Several series of patients with t(8;21) report that granulocytic sarcomas occur in approximately 18% of this population, which is four times the expected incidence in AML. Thirty-seven cases have been previously reported. Although karyotype analyses were not reported in many cases of granulocytic sarcoma in the literature, the vast majority of abnormal karyotypes in patients with AML involved t(8;21). Recent work with a cell line derived from a patient with t(8;21) indicates that such cells are unusually adherent to culture bottles and are aggregable CONCLUSION: Our data suggest that this association is more common than generally recognized and may be specific. Patients with t(8;21) should be observed closely for signs and symptoms of granulocytic sarcoma. These patients may have a less favorable prognosis than other patients with t(8;21). Cooperative oncology groups should retrospectively identify patients with AML and t(8;21) who had a poor outcome to determine if they had a disproportionate incidence of granulocytic sarcoma. If so, aggressive therapy such as bone marrow transplantation may be warranted early in the therapeutic strategy.

Orbital malignant peripheral nerve sheath tumors. Treatment with surgical resection and radiation therapy.

A series of three patients with primary orbital malignant peripheral nerve sheath tumors (MPNST) is presented. Two of our patients who were treated with surgery and postoperative radiotherapy are free of tumor recurrence. The third patient showed a good response to radiation therapy. While surgical excision remains the mainstay of therapy, our patients demonstrate the usefulness of adjuvant radiation therapy in this condition.

Clinical study on the effectiveness of tear drainage with a single canalicular system under environmental stress.

Sixteen asymptomatic volunteers had their right lower puncta occluded with a Freeman silicone plug. They then went about their normal daily activities for approximately 1 week under the stress of a cold Chicago winter. Of the 16 eyes that were plugged, 4 had epiphora. The episodes were of short duration and did not have any effect on the subjects' daily activities. When comparing the incidence of epiphora between the plugged eyes and the control eyes, there was no statistically significant difference (p = 0.082). Also, when comparing the modified Jones I test and Schirmer tests, with and without anesthesia, there was no significant difference in the values between the plugged and the control eyes (p > 0.05). The only difference noted was in the dye disappearance test (p < 0.05). These results support the hypothesis that in selected patients, even under stressful environmental conditions, a single healthy canaliculus may provide sufficient tear drainage from a normal eye to prevent bothersome subjective symptoms.

Surface reaction on silicone tubes used in the treatment of nasolacrimal drainage system obstruction.

Silicone tubes removed from patients who underwent intubation of the nasolacrimal system for acquired or congenital obstruction were studied to determine the cellular reaction on the tubes and to assess the relationship between the length of intubation and the cellular response. Twenty-one tubes were available for analysis. Length of intubation varied from 39 to 415 days; patient ages varied from 10 months to 75 years. All patients had undergone dacryocystorhinostomy or closed intubation of the nasolacrimal system. No patient had clinical signs of infection at the time of tube removal. All tubes were mounted on glass slides and stained with hematoxylin and eosin. The reactions seen, together with the location and type of cells present, were graded by a masked observer. There were varying numbers of inflammatory cells, predominantly polymorphonuclear leukocytes. As the length of intubation increased, the number of inflammatory cells also increased. The proximal portion of the tubing showed the least amount of cellular reaction at all lengths of intubation.

Hemangiopericytoma of the lacrimal sac.

Hemangiopericytomas of the orbit are rare tumors with low malignancy potential. To the authors' knowledge, only three previous cases of these tumors occurring in the lacrimal sac have been reported. The authors report a fourth case. Follow-up was obtained on the first reported case, and the patient in that case had a recurrence after 15 years. These tumors should be totally excised and followed for long periods of time.

Isolated orbital roof fracture with traumatic encephalocele.

Several months after a fall down a flight of stairs, a one-year-old girl developed downward displacement and pulsation of the left eye. Vision and ocular motility were not impaired and the child remained neurologically normal. Computed tomography with metrizamide cisternography demonstrated herniation of brain tissue through a traumatic defect in the roof of the orbit. The orbital rim and other craniofacial bones were intact. Repair of the growing fracture found at operation restored normal appearance without disturbing function. To our knowledge, this is the first documented case of encephalocele associated with an isolated fracture of the orbital roof due to blunt head trauma.

Sphenoid wing meningioma causing a proptosis in a child. Case report.

A sphenoid wing meningioma causing a progressive painless proptosis is reported in a 5-year-old boy. The diagnostic evaluation and surgical management of this case are reported. A review of the literature indicates this is the youngest patient with sphenoid wing meningioma.

Common eyelid and orbital problems.

Several pathologic processes can disturb the complex structures of the eyelids and orbital tissues and the muscles that enable their proper function. Some disorders, such as entropion and ectropion, are usually acquired in later years from laxity of supporting structures. Blepharoptosis has a range of causes, from aging to an intracranial aneurysm, and its severity varies, from mild to almost complete closure of the lid fissures. Graves' orbitopathy can produce lid retraction, lid lag, and proptosis and can cause vision-threatening conditions. Orbital cellulitis produces pain, swelling, proptosis, and redness of the lids and conjunctivae and, if severe, can compromise ocular motility and visual acuity. Early recognition of the specific disorder and prompt and adequate treatment are essential to spare the patient discomfort and ensure preservation of vision.

The morning glory syndrome associated with sphenoidal encephalocele.

A 13-year-old girl was found to have a "morning glory" optic disc anomaly associated with remnants of the primitive hyaloid vasculature, midline cleft lip and palate, agenesis of the corpus callosum, and a sphenoidal encephalocele. The association of these developmental anomalies indicates that the "morning glory" optic disc anomaly may occur as part of a more extensive syndrome of midline cranioencephalic dysraphism.

Pneumatosis oculi and spontaneous hyphema in association with pneumatosis intestinalis.

A premature infant with acute necrotizing enterocolitis, Escherichia coli sepsis, and disseminated intravascular coagulation developed spontaneous bilateral hyphemas at 3 days of age. The necrotizing enterocolitis was associated with gas bubbles in the intestinal walls. The anterior chambers of both eyes also contained bubbles of gas, formed possibly by a mechanism similar to those in the intestine.

Endogenous intraocular Nocardia asteroides in Hodgkin's disease.

A 60-year-old man receiving antituberculous and corticosteroid therapy for a granulomatous disease of uncertain etiology was found to have a chorioretinal mass in his right eye. Fluorescein angiography showed blockage of fluorescence by the mass and late leakage. Autopsy findings were compatible with Hodgkin's disease with disseminated nocardiosis caused by Nocardia asteroides. Organisms typical of Nocardia were found in the choroid and subretinal space. The patient's history, ophthalmic examination, and fluorescein angiographic findings suggested a type of chorioretinal involvement.