Orbital involvement in extranodal natural killer T cell lymphoma: an atypical case presentation and review of the literature.

PURPOSE: To report a rare case of extranodal NK/T cell lymphoma (NKTL) and to compare its features with those cases previously reported. DESIGN: Case report, observational and literature review. METHODS: Complete ophthalmologic examinations followed by excisional biopsy, histopathologic examination and therapy with radiation and chemotherapy. MAIN OUTCOME MEASURES: Evaluation of clinical presenting features and histopathologic diagnosis along with patient outcome. RESULTS: A 22 year old female presented as a referral with right orbital swelling, decreased vision and eye pain for 5 weeks. Subsequent orbital CT and multiple biopsies resulted in a diagnosis of extranodal natural killer (NK)/T cell lymphoma (NKTL). Despite continued chemotherapy and orbital radiation the patient expired within 3 months of diagnosis. To our knowledge, only 8 cases of orbital involvement without nasal mucosal involvement are reported in the literature, the majority in patients of male gender around the fifth decade. CONCLUSIONS: Here we present an atypical and aggressive case of extranodal NK/T cell lymphoma presenting in a 22 year old Caucasian female as orbital swelling without evidence of nasal mucosal involvement. It is important to distinguish NKTL from the more common benign lymphoproliferative lesions of the orbital adnexa as prognosis of these two clinical entities varies and timely diagnosis is key. The present case demonstrates that extranodal NKTL can occur in the orbit without evidence of the more common nasal mucosal presentations and should be included in the differential diagnosis of ocular adnexal lesions suspicious for a lymphoproliferative disorder and/or an inflammatory process.

The surgical pathology and cytopathology of US Persian Gulf War military veterans.

BACKGROUND: Tens of thousands of Persian Gulf War veterans (GWVs) have presented with medical symptoms since Operation Desert Shield and Operation Desert Storm. The Kuwait Registry at the Armed Forces Institute of Pathology was established to act as a repository for surgical pathology, cytopathology, and autopsy material from GWVs. OBJECTIVE: To identify conditions known to be endemic to the theater of operations in our cohort of GWVs. METHODS: The Kuwait Registry database was searched by computer for listed conditions endemic to the Persian Gulf region included in the registry through December 31, 1997. RESULTS: Of the 2582 patients in this cohort, 1 patient with hepatitis B and 15 patients with hepatitis C were identified. Other known endemic conditions of the Persian Gulf region were not found. CONCLUSIONS: Viral hepatitis (B and C), which is prevalent in the US population, was the only listed endemic condition identified in surgical pathology or cytopathology specimens in our cohort of GWVs.

Adenoid cystic carcinomas of the lacrimal gland in childhood and adolescence.

OBJECTIVE: To see if there is a correlation between histologic features of these tumors and final outcome. DESIGN: A small series of cases of adenoid cystic carcinomas of the lacrimal gland in patients 18 years of age or younger were evaluated. PARTICIPANTS: A total of 11 cases of adenoid cystic carcinoma of the lacrimal gland in patients 18 years of age or younger found in the registry of Ophthalmic Pathology at the Armed Forces Institute of Pathology were studied. INTERVENTION: Histologic material obtained by excision of lacrimal gland tumors was evaluated for different morphologic parameters. Clinical follow-up information was reviewed. MAIN OUTCOME MEASURES: All cases were evaluated for proportion of a basaloid histologic pattern: necrosis, hemorrhage, mitotic count, and perineural, vascular, intraosseous, leptomeningeal, and optic nerve invasion. These parameters were examined for an association with the clinical follow-up that was obtained for eight of the patients (mean follow-up, 10 years; range, 2-14 years). RESULTS: Most of the patients were female (M:F = 2:9). Mean age was 14 years (range, 6.5-18 years). Of the patients with follow-up, 5 (62.5%) of 8 survived. Estimated survival rate at 15 years was 58% (Kaplan-Meier analysis). Survivors had 25% or less basaloid histology. Necrosis, hemorrhage, perineural invasion, and mitotic count were less prominent in survivors than in those who died of disease. Vascular invasion was seen only in fatal cases. CONCLUSIONS: Young patients with adenoid cystic carcinomas have a better prognosis than do adult patients, which may be due to their tumors having less aggressive histologic features.

Primary orbital melanomas.

PURPOSE: Primary orbital melanomas are rare tumors with a poorly defined biologic course. Most recorded experiences concern single case reports. The authors evaluated the applicability of several of the histopathologic prognostic indicators used for uveal melanomas to a series of primary orbital melanomas with known clinical follow-up. METHODS: Twenty-one primary orbital melanomas, each with at least a 1-year follow-up after diagnosis, were evaluated for (1) modified Callender cell type, (2) mitotic count per 40 high-power fields, (3) lymphocyte count (less than versus greater than 100/20 high-power fields), (4) blue nevus component, and (5) largest tumor diameter. RESULTS: All patients for whom race was recorded were white. The mean age at diagnosis was 42 years (range, 15-84 years). There was an associated blue nevus in 19 patients (90 percent), and in 10 patients (47.5 percent) there was some form of congenital melanosis. With a mean follow-up period of 4.5 years (range, 1-13 years), mortality from metastatic tumor occurred in 8 (38 percent) of 21 patients. Of these eight patients, there were liver metastases in seven (88 percent) and brain metastases in one (12 percent). Indicators of poor prognosis were tumors of mixed cell type with high mitotic count and greater patient age with underlying congenital melanosis. CONCLUSION: Most primary orbital melanomas occur in white patients and are associated with blue nevi. These tumors are similar to uveal melanomas with respect to prognostic indicators and pattern of metastasis.

Benign orbital fibrous histiocytoma simulating a lacrimal gland tumor.

The case of a patient with a lacrimal fossa mass that was believed to be a primary lacrimal gland tumor is reported. However, at lateral orbitotomy the tumor was found within the lacrimal fossa, but distinct from the lacrimal gland. Histopathology revealed a benign fibrous histiocytoma. The tumor was totally excised, has not recurred over a 3-year follow-up period. This mesenchymal tumor should be included in the differential diagnosis of lacrimal fossa mass in adults.

Oncocytic adenocarcinoma of the lacrimal sac: report of a case with paranasal sinus and orbital extension.

A 92-year-old man has had multiple recurrent oncocytic tumors involving the right paranasal sinuses and orbit. A benign oncocytoma that arose from the right lacrimal sac was initially diagnosed at age 80 years. The tumor recurred at 3 and 7 years after initial resection. There was greater histologic atypia in the first recurrent tumor, which extended into the right paranasal sinuses. The second recurrence had zones on oncocytic adenocarcinoma exhibiting prominent nuclear atypia and mitotic activity; this tumor massively invaded the right paranasal sinuses and orbit. We describe the clinical and pathologic features of this rare case.

Posterior keratoconus. Case report with scanning electron microscopy.

We report the clinical and scanning electron microscopic findings in an older adult patient with unilateral posterior keratoconus. Clinically, the cornea showed typical posterior concavity with focal underlying anterior ectasia and an epithelial iron ring. Scanning electron microscopy of the cornea removed at penetrating keratoplasty revealed a pericentral posterior concavity. The endothelial layer was intact, with moderate endothelial cell pleomorphism. There were no excrescences of Descemet's membrane or endothelial tags. The lack of discrete irregularities on the posterior corneal surface in this case is different from previous descriptions of the pathological appearance of posterior keratoconus, which have been used to support a relationship of this condition to central corneal mesenchymal dysgenesis. This is the first report of scanning electron microscopic findings in posterior keratoconus.

Use of remote video microscopy (telepathology) as an adjunct to neurosurgical frozen section consultation.

We investigated the use of remote video microscopy (telepathology) to assist in the diagnosis of 52 neurosurgical frozen section cases. The TelMed system (Discovery Medical Systems, Overland Park, KS), in which the referring pathologist selects appropriate fields for transmission to the consultant, was used for the study. There was a high degree of concordance between the diagnosis rendered on the basis of transmitted video images and that rendered on the basis of direct evaluation of frozen sections; however, in seven cases there was substantial disagreement. Remote evaluation was associated with a more rapid consultation from the standpoint of the consultant, who spent approximately 2 minutes less per case when using remote microscopy; this was achieved at the expense of considerably greater effort on the part of the referring pathologist, who spent approximately 16 minutes per case selecting an average of 4.5 images for transmission to the consultant. The use of remote video microscopy for pathology consultation is associated with a complex series of tradeoffs involving cost, information loss, and timeliness of consultation.

Orbitocranial wooden foreign body diagnosed by magnetic resonance imaging. Dry wood can be isodense with air and orbital fat by computed tomography.

In computed tomographic (CT) scans, a wooden foreign body can appear as a lucency with nearly the same density as air or fat, and it can be indistinguishable from orbital adipose tissue. Magnetic resonance imaging (MRI) can localize these wooden foreign bodies in the orbit. We studied a case in which a wooden golf tee lodged in the right optic canal of a nine-year-old boy. The head portion lodged in the orbital apex and the tip entered the interpeduncular fossa. Clinical examination revealed a right paranasal laceration; the right eye had no light perception and a peripapillary hemorrhage, but was otherwise normal. Surgical exploration and evaluation by CT failed to locate the foreign body. However, the golf tee was demonstrated by MRI as a low intensity image. Although it was removed by craniotomy with good neurological results, bacterial panophthalmitis led to enucleation of the eye. This case emphasizes the diagnostic value of MRI and the hazards of retained wooden foreign bodies.

Ocular histoplasmosis with retinitis in a patient with acquired immune deficiency syndrome.

Disseminated histoplasmosis is one of the life-threatening opportunistic infections associated with acquired immune deficiency syndrome (AIDS). A 29-year-old man with AIDS and disseminated histoplasmosis complained of a hazy spot in the vision of his left eye. Results of examination showed distinct creamy white intraretinal and subretinal infiltrates in both eyes. The patient died within a month from pulmonary infection with Histoplasma capsulatum and cytomegalovirus. Examination with light microscopy showed that the right and left eyes contained histoplasma yeast cells in lesions of retinitis, optic neuritis, and uveitis. These lesions contained variable numbers of lymphocytes and histiocytes. Electron microscopy of the histoplasma in tissue showed characteristic features. This case illustrates the funduscopic appearance and histopathology of histoplasmic retinitis, an uncommon although important ophthalmologic complication of AIDS.

Immunohistochemical properties of human optic nerve glioma. Evidence of type 1 astrocyte origin.

The peroxidase-antiperoxidase method was used to study ten surgically obtained human optic nerve gliomas (pilocytic astrocytomas). All tissues were formalin fixed and paraffin embedded. Primary antisera included glial fibrillary acidic protein (GFAP), HNK-1 (type 1 astrocyte precursor marker), A2B5 (type 2 astrocyte precursor marker), S-100, vimentin, myelin basic protein (MBP), laminin, keratin, cytokeratin, epithelial membrane antigen (EMA), and neuron-specific enolase (NSE). Neoplastic astrocytes in optic nerve gliomas stained with GFAP, HNK-1, S-100, and vimentin. Oligodendrocytes and myelin sheaths stained for MBP, and NSE stained surviving axons in the tumors. Neoplastic astrocytes did not stain for A2B5, keratin, cytokeratin, EMA, or laminin. These results suggest that human optic nerve gliomas (pilocytic astrocytomas) arise from type 1 astrocytes.

Primary rhabdomyosarcoma of the iris.

We describe herein a 4-year-old girl with primary rhabdomyosarcoma of the right iris. Malignant cells were aspirated from the aqueous humor, and the eye was enucleated 7 months after the tumor appeared. To our knowledge, only two cases of primary rhabdomyosarcoma of the iris have been reported. Both of these patients received radiation therapy prior to enucleation.

Intracapsular lens delivery during attempted extracapsular cataract extraction. Association with capsulorrhexis.

Continuous circular tear capsulotomy, or capsulorrhexis, has become a popular technique in cataract extraction, especially in phacoemulsification. The authors report on six cases from five different surgeons at three institutions, in which the entire lens was inadvertently delivered on attempted expression of the nucleus after circular capsulorrhexis. In one case, scanning electron microscopy suggests that the smooth edge of a small, closed, capsular tear is not capable of controlled extension to allow nuclear egress. Therefore, superior radial anterior capsular relaxing incision is recommended when circular capsulorrhexis is used in extracapsular cataract extraction.

Traumatic enucleation for posterior uveal melanoma.

Two features of eyes enucleated for posterior uveal melanoma that may serve as indicators for traumatic enucleation and relate to dissemination of tumor cells at the time of enucleation are myelin artifact of the optic nerve head and acute hemorrhage within the tumor. Myelin artifact occurs when crushed optic nerve tissue is squeezed into the eye at the time of enucleation. Intralesional hemorrhage may occur during surgery and may be correlated with fluctuations in intraocular pressure. We reviewed 519 cases of posterior uveal melanoma treated by enucleation between 1950 and 1970. Without knowledge of the follow-up data, we examined histologic sections for myelin artifact, intralesional hemorrhage, subretinal hemorrhage, Callender cell type, size of tumor, necrosis, and scleral or orbital invasion. Neither myelin artifact nor intralesional hemorrhage were independent prognostic risk factors. These findings do not support or refute the hypothesis that excessive trauma during enucleation results in a worse prognosis.

Toxoplasma gondii retinochoroiditis and optic neuritis in acquired immune deficiency syndrome. Report of a case.

A 29-year-old man with acquired immune deficiency syndrome (AIDS) was found to have a retinochoroiditis and optic neuritis of his left eye. Results of fundus examination showed inflammatory and hemorrhagic retinal lesions consistent with retinitis due to cytomegalovirus infection. A computed tomographic (CT) scan demonstrated enlargement of the optic nerve. The eye was enucleated and results of histopathologic examination showed retinal necrosis with underlying choroiditis and an optic neuritis. Numerous encysted Toxoplasma gondii organisms were present in the retina and tachyzoites were present in the optic nerve. To the authors' knowledge this is the second histologically documented case of Toxoplasma optic neuritis in a patient with AIDS.

Lymphocytic infiltration in uveal malignant melanoma.

A study relating the intralesional infiltration of lymphocytes and plasma cells to patient survival was performed on cases of uveal malignant melanoma accessed at the Armed Forces Institute of Pathology, Washington, DC (AFIP) between 1954 and 1971. The authors examined 1193 cases using light microscopy. Of the 1078 cases with technically acceptable histologic sections, 134 tumors contained 100 or more lymphocytes per 20 high-power (X400) microscopic fields (20 HPF). The prevalence was 12.4%. This was designated the "high lymphocytic" group. An equivalent number of cases with fewer lymphocytes comprised the "low lymphocytic" group. The survival rate at 15 years was 36.7% for patients in the high lymphocytic group and 69.6% for patients in the low lymphocytic group. Using the Cox model, the authors found that an increased number of lymphocytes per 20 HPF was significantly associated with decreased survival (chi-square = 21.2, P = less than 0.0001). A significant association was observed even when we controlled for other risk factors (chi-square = 6.98, P = 0.008).

Uveal malignant melanoma and von Recklinghausen's neurofibromatosis.

A case of uveal malignant melanoma with extensive spread to the central nervous system occurring in a 63-year-old woman with peripheral-type von Recklinghausen's neurofibromatosis was studied by light microscopy. The tumor histologically consisted of large, loosely coherent epithelioid cells with abundant eosinophilic cytoplasm and large, irregular nuclei with prominent nucleoli. The tumor cells contained variable amounts of melanin pigment and exhibited positive immunohistochemical staining for S-100 protein and neuron-specific enolase. A review of the literature disclosed only ten previous cases of uveal malignant melanoma associated with von Recklinghausen's neurofibromatosis. A predominance of women and of the peripheral variant of von Recklinghausen's neurofibromatosis was noticed in the cases reported.

Spontaneous hemorrhage in a mixed glioma of the cerebellum: case report.

An 8-year-old boy presented in coma and was found to have a massive posterior fossa hemorrhage on computed tomographic scan. Autopsy disclosed a large cerebellar hematoma within a mixed glioma containing both juvenile pilocytic astrocytoma and oligodendroglioma. It is postulated that the hemorrhage originated from the oligodendroglial component of the tumor.

Myxopapillary ependymoma of the filum terminale. A light and electron microscopic study.

The histology of a myxopapillary ependymoma of the filum terminale in a 15-year-old girl was studied by light and electron microscopy. The neoplasm contained both papillary and compact areas of cell growth. The papillary regions consisted of vacuolated cuboidal cells arranged around hyaline/vascular cores that were variably mucin-positive. In the compact regions, the tumor cells were intensely immunoreactive for glial fibrillary acidic protein and occasionally formed perivascular pseudorosettes. Ultrastructural examination revealed interdigitating cell processes, sometimes resting on a basement membrane. Surface specializations included numerous microvilli and occasional cilia. The cilia had an abnormal microtubular pattern (e.g., 8 + 1, 7 + 0). The cytoplasm of the tumor cells contained numerous 7-10 nm intermediate filaments and large numbers of abnormal microtubular aggregates, consisting of 4 to 50 single microtubules enclosed by a limiting membrane. These microtubular aggregates may represent a characteristic feature of myxopapillary ependymoma of the filum terminale.