Retrospective Study of Metastatic Melanoma and Renal Cell Carcinoma to the Brain with Multivariate Analysis of Prognostic Pre-Treatment Clinical Factors.

Patients with brain metastasis from renal cell carcinoma (RCC) or melanoma have historically had very poor prognoses of less than one year. Stereotactic radiosurgery (SRS) can be an effective treatment for patients with these tumors. This study analyzes the effect of pretreatment prognostic factors on overall survival (OS) for RCC and melanoma patients with metastasis to the brain treated with SRS. A total of 122 patients with brain metastases from either RCC or melanoma were grouped by age at brain metastasis diagnosis, whether they received whole brain radiation therapy (WBRT) in addition to SRS, or they underwent surgical resection, Karnofsky Performance Score (KPS), number of brain metastases, and primary tumor. Median survival times for melanoma patients and RCC patients were 8.20 ± 3.06 and 12.70 ± 2.63 months, respectively. Patients with >5 metastases had a significantly shorter median survival time (6.60 ± 2.45 months) than the reference group (1 metastasis, 10.70 ± 13.40 months, p = 0.024). Patients with KPS ≤ 60 experienced significantly shorter survival than the reference group (KPS = 90-100), with median survival times of 5.80 ± 2.46 months (p < 0.001) and 45.20 ± 43.52 months, respectively. We found a median overall survival time of 12.7 and 8.2 months for RCC and melanoma, respectively. Our study determined that a higher number of brain metastases (>5) and lower KPS were statistically significant predictors of a lower OS prognosis.

Bilateral vision loss as initial presentation of chronic myeloid leukemia in a young adult: A case report and review of the literature.

Purpose: To report a case of bilateral vision loss as the primary presenting symptom of chronic myeloid leukemia in a young adult. Observations: The 28-year-old male patient presented to clinic with visual acuity of 20/200 in both eyes after several months of episodic bilateral vision loss. Intraretinal and pre-retinal hemorrhages were appreciated, as well as Roth spots and peripheral neovascularization. Initial lab findings were consistent with a diagnosis of acute myeloid leukemia, later, upon bone marrow examination, the diagnosis was edited to chronic myeloid leukemia. Dasatinib therapy resulted in a complete hematologic resolution after six weeks. After intravitreal injections of bevacizumab in both eyes, visual acuity improved to 20/25 in the right eye and 20/20 in the left eye. Conclusion and importance: After review, this is one of only a few reported cases of bilateral blurry vision as the primary presenting symptom of chronic myeloid leukemia in a young adult. Because visual disturbances occur more frequently in acute myeloid leukemia, and lab results may be inconclusive, careful consideration should be given to differentiate myelogenous leukemias, as the acute and chronic subtypes may present similarly.

Urologic Manifestations and Hydronephrosis as Initial Presentation of Erdheim-Chester Disease: A Rare Form of Non-Langerhans Histiocytosis.

Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis that is classified as a malignancy of myeloid progenitor cells, with only 1,000 confirmed cases in the literature so far. It often manifests as a multi-system disorder with an initial presentation predominantly in the long bones, central nervous system (CNS), and retroperitoneal space, sometimes causing urologic symptoms as a result. ECD often presents indolently and in a spectrum of different ways, making it challenging to identify and treat. We report a case of a 63-year-old female with ECD that first presented with abdominal pain and acute renal injury due to ECD-related retroperitoneal fibrosis. We also explore the literature at large around ECD, its diagnosis, pathophysiology, and advances in treatments.

Single Solitary Fibrous Tumor Brain Metastasis in a Patient with Simultaneous Adenocarcinoma of the Lung: Case Report and Review of the Literature.

We present a unique case of a patient simultaneously diagnosed with solitary fibrous tumor (SFT) and unrelated adenocarcinoma of the lung, both proven with separate pathology. It was subsequently found that the SFT had metastasized to the brain by additional pathology, and not the predicted adenocarcinoma. SFTs are a rare mesenchymal neoplasm that accounts for less than 2% of all reported soft tissue tumors. SFTs most commonly arise in the thoracic cavity, but are frequently found in various locations throughout the body, and rarely metastasize to the brain. This case highlights that rare neoplasms, such as SFT, should not be ruled out as a potential cause of metastasis. Due to the rarity of this clinical situation, we also provide a review and discussion of previously reported SFT cases and the use of postoperative radiation therapy. The optimal treatment for individual patients remains unclear in this unique situation. Surgical resection followed by adjuvant Gamma Knife radiation therapy to the surgical bed appears to be a safe option for local treatment of SFT in select patients. Further studies are needed of this rare clinical situation in order to better understand and optimize future treatments for patients with SFT and metastasis to the brain.

Overall and cause-specific survival for mucoepidermoid carcinoma of the major salivary glands: Analysis of 2210 patients.

BACKGROUND: Mucoepidermoid carcinoma (MEC) is a rare malignancy of the head and neck; however, it accounts for a majority of the tumors of the salivary glands. This study used a national population-based registry to describe the pre-treatment and treatment-related prognostic factors that influence survival in patients with MEC of the major salivary glands. To our knowledge, this is the largest population-based study examining predictors of both overall and cause-specific survival of MEC of the major salivary glands. AIM: To identify prognostic factors influencing overall survival (OS) and cause-specific survival (CSS) of patients with MEC of the major salivary glands. METHODS: We used the Surveillance, Epidemiology and End-Results Database of the National Cancer Institute to investigate a variety of factors that could influence survival of patients diagnosed with mucoepidermoid carcinoma of the major salivary glands. A total of 2210 patients diagnosed with MEC of the major salivary glands during the years of 1975-2016 were studied. The primary endpoints were OS and CSS. Cox regression analysis was used to perform univariate and multivariate analyses of clinical variables such as age at diagnosis, diagnosis year, sex, race, tumor size, stage, grade, treatment with or without surgical excision, and adjuvant radiotherapy treatment. RESULTS: A total of 2210 patients diagnosed with MEC of the major salivary glands met inclusion criteria. In this study, 95% of patients underwent surgical excision and 41% received adjuvant radiation therapy. Median OS time for Grade I, II, and III/IV was 401 mo (± 48.25, 95%CI), 340 mo (± 33.68, 95%CI) and 55 mo (± 11.05, 95%CI), respectively. Univariate analysis revealed that lack of surgical excision was associated with decreased OS [hazard ratio (HR) 4.26, P < 0.0001] and that patients with localized disease had improved OS compared to both regional and distant disease (HR 3.07 and 6.96, respectively, P < 0.0001). Additionally, univariate analysis demonstrated that male sex, age over 50 at diagnosis, Grade III tumors, and increasing tumor size were associated with worsened OS (P < 0.0006). Univariate analysis of CSS similarly revealed that lack of surgical excision and Grade III carcinoma conferred decreased CSS (HR 4.37 and 5.44, respectively, P < 0.0001). Multivariate analysis confirmed that increasing age, in 10-year age bands, advanced tumor stage, increasing tumor size, Grade III carcinoma, male sex, and lack of surgical excision were associated with a statistically significant decrease in OS and CSS (P < 0.04). Of note, multivariate analysis revealed that the use of adjuvant radiation therapy was not associated with improved OS or CSS. CONCLUSION: Multivariate analysis demonstrated increasing age, advanced tumor stage, increasing tumor size, Grade III carcinoma, male sex, and lack of surgical excision were associated with decreased OS and CSS (P < 0.04).

Clinicopathologic Features and Survival Trends for Acinic Cell Carcinoma of the Major Salivary Glands: A Surveillance, Epidemiology, and End Results Population Analysis.

BACKGROUND: We analyzed a population-based national registry to identify the most influential patient pretreatment and treatment factors affecting overall survival (OS) and cause-specific survival (CSS) in patients diagnosed with acinic cell carcinoma (ACC) of the major salivary glands. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) database of the US National Cancer Institute (NCI) related to survival, a total of 1,254 patients with diagnosed ACC of the major salivary glands from 1975 to 2016 met inclusion criteria. Factors significant for OS and CSS were determined using univariate and multivariate analysis with the Cox proportional hazards model. RESULTS: Univariate OS analysis demonstrated that surgery favorably influenced longer survival compared to no surgery (hazard ratio (HR) 2.35, P < 0.05). Patient age was found to be highly predictive of superior OS (divided into 10-year age bands, P < 0.0001, younger age better). In multivariate OS analysis, there were statistically significant worse outcomes for men (HR 1.54, P < 0.05), grades III/IV (HR 2.5, P < 0.05), and distant disease (HR 3.55, P < 0.05) or regional disease (HR 1.22, P < 0.05). Patients diagnosed during years 1996 - 2016 had better OS when compared to earlier decades 1975 - 1995 (HR 1.38, P < 0.05). In univariate analysis, the mean CSS for grades I, II, and III/IV were 429 months (95% confidence interval (CI), ± 38.39), 426 months (95% CI, ± 25.73) and 198 months (95% CI, ± 66.38). Multivariate analysis of CSS further demonstrated that there were statistically significant worse outcomes for men (HR 1.68, P < 0.05), grade III/IV (HR 3.2, P < 0.05), tumor size greater than 40 mm (P < 0.001), and distant disease (HR 4.48, P < 0.05) or regional disease (HR 1.84, P < 0.05). CONCLUSIONS: To our knowledge, this is the largest population-based study of OS and CSS of major salivary gland ACC. We found that the patient pretreatment and treatment factors including younger age at diagnosis, female sex, early stage, lower grade, surgical excision, and recent year of diagnosis are associated with improved survival in patients diagnosed with ACC of the major salivary glands. We hope that this information will aid in construction of further research projects that better refine optimal treatment protocol of individualized patient care.

Primary Epithelioid Sarcoma of Orbit: A Case Report and Review of the Literature.

Epithelioid sarcoma is a rare high-grade malignancy identified by Enzinger in 1970. It accounts for 1% of all reported soft tissue sarcomas and presents most commonly in distal upper extremities in young adults with a male predominance. At this time, there are only 5 previously reported cases of primary epithelioid sarcoma of the orbit. We present a primary orbital epithelioid sarcoma case of a patient who underwent orbital exenteration followed by external beam radiation treatment. Because the literature is limited, this is to our knowledge the largest descriptive analysis of cases of orbital epithelioid sarcoma. We also provide a detailed review of all the previously reported primary orbital epithelioid sarcoma cases, as well as a discussion on the use of postoperative radiation therapy for patients with epithelioid sarcoma. Surgical resection followed by adjuvant radiation therapy appears to be a safe option for local treatment of this rare malignancy, but further future studies are needed of this rare clinical situation in order to better understand and optimize treatment for patients with orbital epithelioid sarcoma.