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A broad spectrum of lymphomas can affect the eye and orbital tissues. This article discusses those that affect the orbit, lids, lacrimal gland, and conjunctiva.
PURPOSE: To review the clinical, radiological, and histopathologic features in 8 patients with natural killer/T-cell lymphoma (NKTL) involving the orbit and/or ocular adnexa, and to describe the responses of these patients to various treatment regimens. DESIGN: Retrospective observational case series. PARTICIPANTS: Eight patients (5 male, 3 female) with NKTL involving the orbit and/or ocular adnexa were identified from 1999 through 2005. The mean age at presentation was 45 years (range, 26-65). METHODS: We retrospectively identified patients with NKTL of the ocular adnexa treated in the authors' medical centers from 1999 through 2004 using computerized diagnostic index retrieval. The clinical records and radiologic studies were analyzed to define modes of presentation and progression, response to therapy, and areas of anatomic involvement. Histopathologic findings, including the presence of CD3, CD56, and Epstein-Barr virus-encoded mRNA in each patient, were reviewed. MAIN OUTCOME MEASUREMENTS: Time of survival from presentation to last known follow-up and tumor-related death. RESULTS: Four of the 8 patients (50%) with NKTL involving the orbit or ocular adnexa had systemic involvement at presentation. Five of the 8 patients (62.5%) had concurrent sinonasal involvement, whereas 3 (37.5%) had orbital involvement alone. All lesions demonstrated CD3, CD56, and/or Epstein-Barr virus positivity on immunopathology studies. Therapy consisted of various chemotherapeutic regimens typically employed in the treatment of non-Hodgkins lymphoma, steroids, surgical intervention, and radiation. Seven (87.5%) patients died 5 weeks to 13 months after presentation, and 1 (12.5%) is alive without disease (5-year follow-up). CONCLUSIONS: Natural killer/T-cell orbital lymphoma is a rare Epstein-Barr virus-associated neoplasm that may occur with or without associated sinonasal involvement. Our series, the largest cohort reported to date, demonstrates the high lethality of this condition despite aggressive conventional therapy, suggesting that new treatment options should be considered early in the course of treatment of patients with this disorder.
BACKGROUND: Schwannoma is a well-defined, usually benign tumor arising from the Schwann cells in the nerve sheath. It can present in any location as a solitary mass; however, it is rarely found in the pelvis. CASE: We report a 40-year-old female patient with a pelvic mass, which revealed tubal schwannoma after complete resection of the mass and uterus by laparotomy. CONCLUSION: Schwannomas are thought to result from a proliferation of perineural cells. Although origin along the nerves of the retroperitoneal space is not uncommon, these tumors rarely present as pelvic masses.
BACKGROUND: Small cell lung cancer (SCLC) carries a very poor prognosis, although recent advances in chemotherapy have produced some long-term survivors. Despite this, recurrences are common, and these patients require a high level of surveillance. Given that the female gender is a favorable prognostic factor in patients with SCLC and that more women are becoming lung cancer survivors, it is important to consider sites of metastasis that are restricted to women. CASE: This is a presentation of a 42-year old previously diagnosed with SCLC who presented with an isolated recurrence of SCLC in the ovaries. This is a case report of an isolated recurrence of SCLC in the ovaries with a review of the literature. CONCLUSION: Although systemic relapses and recurrences often occur in these patients, isolated metastasis such as this can occur and should be considered in female patients with a history of small cell lung cancer.
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OBJECTIVE: To determine whether perioperative packed red blood cell (PRBC) and fresh frozen plasma (FFP) transfusions during ovarian, tubal, or peritoneal cancer surgery increase the risk of symptomatic postoperative venous thromboembolism (VTE) and adversely affect overall survival. METHODS: We conducted a retrospective review of all cases of surgical exploration for resection of stage IIIC-IV adnexal/peritoneal cancer between November 1998 and May 2002 at Memorial Sloan-Kettering Cancer Center. Patients with a history of prior or active preoperative VTE were excluded. Routine intraoperative and postoperative VTE prophylaxis including lower extremity external pneumatic compression with or without postoperative subcutaneous heparin was utilized in all cases. Symptomatic postoperative VTE was diagnosed by lower extremity Doppler or computerized tomography (excluding cases with only ovarian vein thrombosis). Clinical parameters were examined by a logistic regression analysis to identify independent prognostic predictors of postoperative symptomatic VTE, which occurred within 30 days of surgery. Survival was calculated using the Kaplan-Meier method. RESULTS: In all, 174 patients underwent exploratory surgery, and 6 (3.4%) were excluded due to active or prior history of VTE. Of the remaining 168 patients, 71 (42%) received at least one perioperative transfusion of PRBC or FFP. Postoperative VTE was documented in 5 of 46 (11%) patients who received a postoperative transfusion compared to 3 of 122 (2.5%) patients who did not (P = 0.04; odds ratio, 4.8); moreover, VTE was noted in 3:16 (19%) patients who received postoperative FFP compared to 5:152 (3.3%) patients who did not (P = 0.01, odds ratio of 6.78). Age, stage, body mass index, length of the operation, blood loss, presence of ascites, volume of ascites, residual disease status, preoperative hemoglobin level and coagulation profile were not associated with increased risk for VTE. When survival results were stratified by transfusion utilization and controlling for optimal debulking status, perioperative transfusions had no apparent effect on overall survival. CONCLUSION: In women with stage IIIC-V disease, postoperative blood product transfusions particularly FFP were associated with increased risk of DVT and PE, but transfusions had no impact on overall survival.
OBJECTIVE: The primary objective was to investigate the occurrence rates of benign and malignant ovarian tumors associated with pregnancy among women identified in three large California databases between 1991 and 1999. The secondary objective was to determine maternal and perinatal outcomes among these pregnancies. METHODS: This is a population-based study of 4,846,505 obstetrical patients using California hospital discharge records from 1991-1999. The California vital statistics birth/patient discharge database was linked to the California Cancer Registry (CCR). Cases of maternal ovarian cancers and low malignant potential (LMP) tumors were separated into three periods based on the timing of diagnosis and pregnancy: prenatal, at delivery, and postpartum. International Classification of Diseases, Revision 9 (ICD-9) codes were used to identify both diagnostic and procedural factors occurring during hospitalizations. The CCR database was used to identify cancer outcomes such as stage, histology, treatments, and vital status. RESULTS: 9375 women had a hospital diagnosis of an ovarian mass associated with pregnancy. CCR database identified 87 ovarian cancers and 115 LMP tumors in the same cohort. The occurrence rates were 0.93% (87/9375) ovarian cancers per total number of ovarian masses diagnosed during pregnancy, and 0.0179 ovarian cancers per 1000 deliveries. The summary stages of the ovarian cancers and LMP tumors were (respectively): localized 65.5% and 81.7%, regional 6.9% and 7.8%, remote 23.0% and 4.4%, and unknown 4.6% and 6.1%. 34 of the 87 ovarian cancers were germ cell tumors (GCT). Malignant ovarian tumors increased the likelihood of maternal outcomes such as cesarean delivery, hysterectomy, blood transfusions, and prolonged hospitalization compared to noncancer pregnant controls, but did not adversely affect neonatal outcomes. Cause-specific maternal mortality of patients with follow-up was 4.7% (9/191) at a mean of 2.43 years after diagnosis. CONCLUSIONS: Ovarian malignancies are rare during pregnancy. Most maternal malignant ovarian neoplasms are early stage and associated with favorable maternal and neonatal outcomes. The low maternal mortality rate is likely due to the predominance of GCTs among the ovarian cancers.
The position of the uterus and ovaries can be affected by the degree of bladder filling. A postvoid scan may bring into view ovarian pathologic conditions not previously discerned.
Because serum CA-125 concentrations are increased in women with endometriosis, our objective was to determine whether CA-125 levels could be used to differentiate endometriotic from nonendometriotic benign cysts. During a 2-year period, all women who had an endometrioma (n = 33) or a nonendometriotic cyst (n = 27) that was greater than or equal to 4 cm were included. Serum CA-125 levels were measured by an immunoradiometric assay, and the mean concentrations in women with an endometriotic cyst increased as the diameter of the cyst increased. The mean CA-125 concentrations in women with cysts greater than or equal to 4 cm were 53 +/- 2 U/ml in 19 women with an endometriotic cyst, 11 +/- 1 U/ml in 20 women with a nonendometriotic cyst, and 15 +/- 1 U/ml in 7 women with a nonendometriotic cyst and endometriosis. In the fluid of 10 endometriomas, the mean CA-125 concentration was 290 +/- 94 X 10(3) U/ml (range 10 to 900 X 10(3) U/ml). With cysts diameters greater than or equal to 4 cm, 19 of 19 women with endometriotic cysts, zero of seven women with nonendometriotic cysts and endometriosis, and zero of 20 women with nonendometriotic cysts had CA-125 concentrations greater than or equal to 20 U/ml (99% upper normal limit). We conclude that serum CA-125 determinations offer an excellent method to differentiate endometriotic from nonendometriotic benign cysts.
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PURPOSE: To report a case of granulocytic sarcoma involving the eyelids and caruncles after bone marrow transplantation. METHODS: Case report. A 30-year-old man with acute myeloid leukemia in remission developed multiple friable eyelid and caruncular lesions in addition to two cutaneous lesions on the chest wall and right axilla approximately 3 months after a successful autologous bone marrow transplant. RESULT: Pathologic examination was consistent with granulocytic sarcoma. CONCLUSION: This condition should be considered in the differential diagnosis of cutaneous or eyelid masses in patients with a history of leukemia.
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OBJECTIVES: To determine cyst fluid and serum vascular endothelial growth factor (VEGF) concentrations in patients with ovarian masses and to investigate the efficiency of this modulator in the clinical management of cystic pelvic masses. METHODS: Needle puncture for cyst fluid aspiration were performed on 88 cystic ovarian masses intraoperatively. Forty-five patients with benign and 43 patients with malignant ovarian pathology were analyzed for cyst fluid and serum VEGF concentrations. Both cystic fluid and serum VEGF concentration were determined by enzyme-linked immunosorbent assay (ELISA). RESULTS: Cyst fluid VEGF levels of malignant cysts (40.65+/-17.69 ng/ml) were significantly higher than those of benign cysts (12.53+/-6.13 ng/ml; P<0.001). Similarly, higher serum VEGF concentrations were found in patients with malignant disease (0.72+/-0.17 ng/ml) compared with benign cysts (0.33+/-0.11 ng/ml; P<0.001). A statistically significant correlation was observed between cyst fluid and serum VEGF levels in both malignant and benign cysts. For serum VEGF, at a cut-off value of 0.41 ng/ml; sensitivity, specificity, PPV, and NPV were 95%, 78%, 80% and 95%, respectively. No significant correlation between cyst fluid VEGF concentration and tumor stage or grade could be found. CONCLUSIONS: Significantly higher concentrations of VEGF are present in cyst fluid and serum of patients with malignant ovarian cysts compared with benign ovarian ones. There is no relation between VEGF and tumor stage or grade.
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Fourteen patients with well- or intermediately differentiated monoclonal B-lymphocytic tumors of the conjunctiva or orbit had a favorable prognosis with follow-ups of 4 to 9 years (mean and median, 7.5 years). The lesions were, for the most part, diffuse proliferations of small lymphocytes, either with round or minimally indented nuclear outlines. Mitotic activity was sparse to nonexistent; occasionally there were scattered small abortive or residual germinal centers, and some lesions exhibited lymphoplasmacytoid features and dispersed multinucleated giant cells (polykaryocytes). None of the six patients with conjunctival lesions had extraocular manifestations. An identical tumor of the submandibular gland developed in one of eight patients with orbital lesions and another patient had multiple extranodal involvements of the oropharynx, liver, and both kidneys, but after chemotherapy the patient has survived for 8 years from orbital presentation and is currently in remission. The authors believe that these low-grade tumors share many biologic resemblances to extranodal lymphoepithelial tumors of other organs (lung, gut, parotid, thyroid), which as a group have been aggregated together as mucosa-associated lymphoid tumors (MALT) and which can often remain localized to their sites of origin.
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