High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review.

BACKGROUND: Spheno-orbital meningiomas (SOMs) are often benign. The association of meningioma and systemic lupus erythematosus (SLE) is rarely discussed in the literature. Here, we report two patients with high-grade, SOMs with a prolonged history of SLE and review the literature. CASE DESCRIPTION: The first case is a 52-year-old female patient with a 15-year history of SLE diagnosis who was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. This patient was operated for the left SOM with histopathological diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma. The second case is a 36-year-old female patient with a 12-year history of SLE diagnosis who presented to our clinic with a 5-year-history of progressive right eye proptosis and occasional headaches. She was operated for the right SOM with histopathological diagnosis of the WHO Grade II chordoid meningioma. CONCLUSION: Rhabdoid and chordoid SOMs are uncommon and no previous report discussed their occurrence in patients with SLE. The association of high-grade meningiomas and SLE deserves further exploration.

Radiation Induced Multiple Skin Neoplasms Following Craniospinal Irradiation for Medulloblastoma. A Case Report.

BACKGROUND Following craniospinal irradiation in children with medulloblastoma, secondary neoplasms are among the most serious long-term sequelae that include leukemias and solid tumors of the urinary or digestive tracts, thyroid, skin, and central nervous system. Furthermore, in children with Gorlin syndrome following craniospinal irradiation for medulloblastoma, there is a rising incidence of skin and non-skin malignancies. CASE REPORT The patient in the present study was a 19-year-old female who was treated with craniospinal irradiation and chemotherapy following gross total resection (GTR) for medulloblastoma at the age of 4 years. Fifteen years later, she developed a primary adnexal tumor at the medial aspect of her left thigh, glomangioma at the skin of her upper abdomen, dermatofibrosarcoma protruberans at the skin of her upper back, and Kaposiform hemangioendothelioma of the upper abdomen. All these tumors were successfully managed with radical resection without further adjuvant treatment. CONCLUSIONS Metachronous of development of 4 histopathologically different skin tumors following craniospinal irradiation for medulloblastoma in long-term survivors has not previously been reported. The present case warrants a detailed dermatological periodic inspection in such patients.

Primary Intracranial Angiomatoid Fibrous Histiocytoma: Two Case Reports and Literature Review.

BACKGROUND: Angiomatoid fibrous histiocytoma (AFH) is a rare, fibrohistiocytic, soft-tissue neoplasm. Intracranial AFH is extremely rare. Here we present 2 pediatric cases of intracranial AFH and perform a literature review on this disease entity. CASE DESCRIPTION: We present 2 cases. The first case is a 10-year-old boy who presented with seizures and hemiparesis. The second case is an 11-year-old girl who presented with 2-year history of seizures. Radiologic images demonstrated right frontal lesions in both patients. Complete surgical resection was achieved. Histopathological findings established the diagnosis of intracranial AFH confirmed with fluorescence in situ hybridization and reverse transcriptase polymerase chain reaction testing that demonstrated EWSR1 gene rearrangement in both cases. CONCLUSIONS: Twenty-two cases of intracranial AFH have been previously documented, with the majority of lesions located in the frontal lobe. Most cases occurred in adolescents and young adults, with a slight female predilection. Headaches and seizures constituted the most common clinical presentation. Complete surgical resection remains the standard of care in the management of this pathology.

Inguinal lymph node presenting as the delayed site of metastasis in early stage endometrial carcinoma: Case report.

INTRODUCTION: Inguinal lymph nodes are the frequent sites of metastasis for malignant lymphoma, squamous cell carcinoma of anal canal, vulva and penis, malignant melanoma and squamous cell carcinoma of skin over lower extremities or trunk. Anatomically, endometrial carcinoma is less likely to spread to the superficial or deep inguinal lymph nodes, thus metastatic involvement of these lymph nodes can easily be overlooked. CASE PRESENTATION: Here-in we report a case of a 65-year old Saudi morbid obese female, who presented with left inguinal lymphadenopathy as initial delayed site of metastasis almost 19 months after the initial treatment for FIGO IA endometrial carcinoma. Patient underwent left inguinal lymph node dissection. Histopathology confirmed metastatic endometrial adenocarcinoma, positive for cytokeratin (CK-7), estrogen receptor (ER) and progesterone receptors (PR), negative for CK-20 and CDX2. Following the post-surgery recovery, she was given extended field radiation therapy to para-aortic, pelvis and bilateral inguinal lymph nodes with concurrent cisplatin chemotherapy followed by high dose rate brachytherapy. CONCLUSION: Inguinal lymph nodes as delayed site of metastasis in early endometrial carcinoma is extremely rare entity. Incorporation of FDG-PET during the preoperative screening of inguinal nodes may be helpful. The impact of lymph node dissection and adjuvant radiation therapy on survival needs to be established.

"Clinicopathological features and treatment outcomes of differentiated thyroid cancer in Saudi children and adults".

INTRODUCTION: Age is an important prognostic factor in differentiated thyroid cancer (DTC). Our aim was to evaluate differences in clinicopathological features and treatment outcomes among children and adult patients with DTC. MATERIALS AND METHODS: We studied 27 children (below 18 years) with DTC treated during the period 2000-2012 and were compared with (a) 78 adults aged 19-25 years and (b) 52 adults aged 26-30 years treated during the same period in terms of their clinicopathological features and long term treatment outcomes. Locoregional recurrence (LRR), locoregional control (LRC), distant metastasis (DM), distant metastasis control (DMC), disease free survival (DFS) and overall survival (OS) rates were evaluated. RESULTS: Mean age of children was 13.5 years (range: 5-18), while mean age of adults was 24.6 years (range: 19-30). In children, female: male ratio was 2.85:1, and in adults female: male ratio was 7.1:1 (P = 0.041). No significant difference in tumor size was seen between the two groups (P = 0.653). According to American Thyroid Association (ATA) risk stratification classification, the children (85.2 %) were found to have at high risk as compared to adults P = 0.001. Post-thyroidectomy complications and RAI induced toxicities were observed more in children than adults (P = 0.043 and P = 0.041 respectively). LRR occurred in 6 (22.2 %), 9 (11.5 %) and 3 (5.8 %) in age groups of <18 years, 19-25 years and 26-30 years respectively (P = 0.032); while DM was seen in 10 (37.0 %), 9 (10.3 %) and 5 (9.6 %) in age groups of <18 years, 19-25 years and 26-30 years respectively (P = 0.002). Ten year DFS rates were 67.3 % in age group below 18 years, 82.4 % in age group of 19-25 years and 90.1 % in age group of 26-30 years (P = 0.021). CONCLUSION: At the time of diagnosis, children with DTC were found to have more aggressive clinicopathological characteristics. Comparatively lower LRC, DMC and DFS rates in children warrants further multi-institutional studies.

Cerebellum as Initial Site of Distant Metastasis from Papillary Carcinoma of Thyroid: Review of Three Cases.

Background. The cerebellum as initial site of distant metastasis from differentiated thyroid carcinoma (DTC) including papillary (PTC) and follicular thyroid carcinoma (FTC) is rare manifestation. Case Presentations. Herein, we present three cases of cerebellar metastasis (CBM) of PTC. Mean age of patients was 67 years (range: 64-72), and mean duration between initial diagnosis and CBM was 49.6 months (range: 37-61). Frequent location was left cerebellar hemisphere and was associated with hydrocephalus. All patients underwent suboccipital craniectomy, and in two patients postoperative intensity modulated radiation therapy (IMRT) was given to deliver 5000 cGy in 25 fractions to residual lesions. Patient without postoperative IMRT had cerebellar recurrence along with lung and bone metastasis after 38 months. However, two patients were found alive and free of disease at the time of last follow-up. Conclusion. CBM from PTC is a rare clinical entity and is often associated with hydrocephalus. Histopathological diagnosis is important to initiate effective treatment, which relies on multidisciplinary approach to prolong the disease-free and overall survival rates.

Pancreas as delayed site of metastasis from papillary thyroid carcinoma.

Introduction. Follicular variant (FV) papillary thyroid carcinoma (PTC) has aggressive biologic behavior as compared to classic variant (CV) of PTC and frequently metastasizes to the lungs and bones. However, metastasis to the pancreas is extremely rare manifestation of FV-PTC. To date, only 9 cases of PTC have been reported in the literature. Pancreatic metastases from PTC usually remain asymptomatic or manifest as repeated abdominal aches. Associated obstructive jaundice is rare. Prognosis is variable with reported median survival from 16 to 46 months. Case Presentation. Herein we present a 67-year-old Saudi woman, who developed pancreatic metastases seven years after total thyroidectomy and neck dissection followed by radioactive iodine ablation (RAI) for FV-PTC. Metastasectomy was performed by pancreaticoduodenectomy followed by sorafenib as genetic testing revealed a BRAF V600E mutation. She survived 32 months after the pancreatic metastasis diagnosis. Conclusion. Pancreatic metastases are rare manifestation of FV-PTC and are usually sign of extensive disease and conventional diagnostic tools may remain to reach the diagnosis.

Bone marrow-derived microglia in pilocytic astrocytoma.

Tumour associated macrophages (TAMs) are increasingly recognized as supporters of tumour growth. The present study was undertaken to examine benign pilocytic astrocytomas (PAs) for the presence of M2 macrophages. We have asked the question whether TAMs in PAs share the predominant CD163 immunophenotype with tumour-associated microglia/macrophages of malignant gliomas. In addition, we were interested in the question whether there is evidence that the macrophages in PAs derive from resident microglia in surrounding normal brain or whether cells expressing a macrophage phenotype may invade PAs from the vasculature. The latter question is of great interest with regard to so-called "bone marrow-derived microglia" (BMDM) which may provide a physiological route of entry into the CNS that could be used for novel cell-based treatments of brain cancer. In fact, we have found strong morphological evidence for such macrophage recruitment into PAs. We propose therefore that PAs may be used as a model for the study of macrophage recruitment into gliomas. Importantly, our results also confirm that microglia/macrophage infiltration per se is not associated with malignant glioma behaviour.