Atypical teratoid/rhabdoid tumor of the central nervous system: Clinicopathological features of two challenging cases.

INTRODUCTION: Atypical Teratoid/Rhabdoid Tumor (AT/RT) is a rare aggressive neoplasm that mainly affects the pediatric population with a peak incidence in the first two years of life and a slight male predominance, whereas presentation of this neoplasm in older ages is extremely rare. CASE PRESENTATION: Herein, we present two cases of AT/RT. In the first case, a 9-year-old female presented with diplopia, dizziness, headache, and morning vomiting. CT Scan of the head demonstrated a heterogeneous mass in the left frontal-parietal region with vasogenic edema and midline deviation. In the second case, a 57-year-old female presented with severe generalized headache, numbness, and tingling in the right hand. MRI revealed a lobulated cystic mass in the right occipitotemporal region, with surrounding edema compressing the left lateral ventricle and causing a midline shift to the left, and enlargement of the right lateral ventricle. In both case, histopathological and immunohistochemical examinations revealed the diagnosis of Atypical teratoid/Rhabdoid tumors. CLINICAL DISCUSSION: Microscopic examination demonstrated the proliferation of medium-sized to large cells with abundant eosinophilic cytoplasm, large vesicular eccentric nuclei, and conspicuous nucleoli with areas of necrosis and hemorrhage, thus confirming the diagnosis with adequate immunohistochemical staining. The first patient developed signs of recurrence and passed away six months later, whereas in the second case, the 57-year-old female received radiotherapy for 6 weeks before being put on chemotherapy. CONCLUSION: Despite the challenges facing the diagnosis of this aggressive neoplasm, we managed to present our cases with detailed histopathological and immunohistochemical examinations.

Large tumoral pseudoangiomatous stromal hyperplasia with ER/PR stromal negativity in a 20-year-old female: A rare case report.

Pseudoangiomatous stromal hyperplasia (PASH) is a rare lesion of the breast stromal tissue with unknown mechanism. Hormonal stimulation of mammary myofibroblasts is the most important theory due to stromal positivity of progesterone receptor (PR) or/and estrogen receptor (ER). We report a case of PASH with stromal PR/ER negativity.

Eccrine spiradenoma on the anterior forearm in a pediatric patient: A rare case report.

Spiradenoma is a rare benign adnexal tumor arising from eccrine sweat glands. It presents as a painful bluish nodular lesion which occurs rarely over the arms. This case report adds to a case where a recurrent spiradenoma occurred over the arm. The majority of reported cases involve young adults, making our pediatric case particularly unusual. Early diagnosis through excisional biopsy is crucial for optimal management and prevention of recurrence or malignant transformation. Eccrine spiradenoma is an uncommon tumor that should be kept in mind in case of a patient with a painful nodule. More genetic studies are needed for the best understanding of this rare tumor entity.

Plasma cell leukemia in a 34-year-old male: rare scenario case report.

Introduction: Plasma cell leukaemia is an uncommon plasma cell dyscrasia with a very poor prognosis. It is more common among males and usually presents between 55 and 65 years of age. Case presentation: A 34-year-old male presented to Al-Assad hospital with unremitting back pain. He was given analgesics but his pain was unresponsive to treatment, and due to the COVID-19 pandemic, he refused a computed tomography scan in the hospital. Later that year, he presented again with weight loss, nausea, abdominal pain, melena, and ascites. He was pale with a moderately distended abdomen. Laboratory tests revealed anaemia, thrombocytopenia, hypercalcemia, increased total proteins, and elevated lactate dehydrogenase. Flow cytometry findings of the bone marrow aspirate showed the presence of 30% of plasma cells, positive for CD38, CD56, and kappa light chains. He was diagnosed with secondary plasma cell leukaemia and started on chemotherapy; however, he could not continue his treatment due to myeloid inhibition. He passed away 5 months later. Clinical discussion: Multiple myeloma was not suspected in the patient due to his young age. The diagnosis was delayed even further due to the COVID-19 pandemic. His multiple myeloma progressed into secondary plasma cell leukaemia and had uncommon features like small intestinal polyps. Even though there has been groundbreaking advancements in chemotherapy, plasma cell leukaemia still possesses a fatal prognosis. Conclusion: This report showcases a rare age presentation with unique manifestations of secondary plasma cell leukaemia. Multiple myeloma should be a differential diagnosis for cases with unexplained back pain despite an unclassical age.

Optimization of tissue microarray technique for breast cancer patients: a short communication.

Background: Tissue microarray (TMA) is a novel technique for studying different types of cancer tissues in one block. TMA is not yet established in Syria, so we aimed in this project to apply and set the most optimal conditions of TMA creation of breast cancer tissues at the Pathology Department of our institute. Materials and Methods: Eighty-eight blocks of breast cancer tissues were selected, considering the inclusion criteria. The tissue specimens of breast cancer patients were manually placed in the block by punching a core from a paraffin block, which was then released into a recipient block using a small trocar. Three different conditions were tested on the constructed TMA block. Results: We determined the most effective parameters that proved high quality: incubating the newly constructed block at a temperature of 43°C for 24 h in the oven and then cutting it the next day after cooling it to room temperature; also, cutting with a 5 µm thickness created the preferable stained slides later. CD3 staining showed high expression of tumor-infiltrating lymphocytes among triple-negative breast cancer patients and high expression of CD3 in triple-negative cancer patients. Conclusion: The optimization of parameters presented in our study resulted in perfect TMA generation and successful immunohistochemistry staining for cancer research at our institution.

Metastatic squamous cell carcinoma of the lung to the thyroid gland presented as nodular goiter: a rare case report.

Metastasis of squamous cell lung carcinoma to the thyroid gland is an extremely rare event. It frequently metastasizes to lymph nodes, liver, adrenal glands, bone, brain, and pleura. Among the lung carcinomas metastasizing to the thyroid, adenocarcinomas are the most common followed by squamous cell carcinomas (SCCs). Case presentation: A 58-year-old male patient presented with bilateral neck swelling. Fine needle aspiration is performed and was undetermined. Ultrasonography of the neck demonstrated multiple hypoechoic nodules with thyroid enlargement. The patient was diagnosed with nodular goitre and he underwent a total thyroidectomy. Microscopically, the Hematoxylin and eosin-stained sections revealed thyroid follicles with sheets composed of polygonal cells with pleomorphic nuclei, prominent nucleoli, and a moderate amount of eosinophilic cytoplasm. Keratin pearls were present. Based on histopathological and clinical findings, the final diagnosis was metastatic SCC to the thyroid gland. Clinical discussion: Clinically, patients with thyroid metastasis presented with nonspecific symptoms such as thyroid nodule or goitre, cervical discomfort, dyspnoea, dysphagia, or dysphonia. Chemotherapy is used in the case of a poly metastatic tumour and radiotherapy as a palliative treatment whereas radioiodine treatment is not indicated for thyroid metastases. Conclusion: Diagnosis of SCC in the thyroid gland as a primary or metastatic neoplasm is a significant challenge. In the absence of specific clinical or radiological signs, pathological studies remain the gold standard for diagnosis.

Whorling-sclerosing meningioma invading skull bone and subcutaneous tissue with an incidental toxoplasmosis: A case report.

Whorling-sclerosing meningioma (WSM) is a rare type of meningothelial tumor. Worldwide, only 31 cases have been reported. The diagnosis of this rare meningioma subtype is based principally on microscopic findings, wherein up to 70% of the tumor tissue should be formed by thick whorls of collagen. Notably, there is still disagreement about whether to consider this entity a benign meningioma (grade 1) or a meningioma with higher malignant potential (grade 2 or even grade 3). In fact, the paucity of reported WSM cases and, consequently, the lack of information about their follow up make their diagnosis and treatment challenging. Herein, we document the first case of WSM in Syria in a 75-year-old woman with an incidental finding of Toxoplasma gondii. Although WSM has not been mentioned in the recent World Health Organization classification of central nervous system tumors, it should be recognized to avoid incorrect diagnosis and spare the patients unnecessary radiotherapy and chemotherapy.

Synchronous ovarian epidermoid cyst torsion with appendicitis in a 2.5-year-old girl: Case report.

INTRODUCTION AND IMPORTANCE: Acute appendicitis associated with ovarian epidermoid cyst torsion is extremely rare. To our knowledge, there are about 7 cases that have been reported in English literature, and there was no epidermoid cyst in any of them. CASE PRESENTATION: Herein, we present the case of a 2.5-year-old girl with a history of vomiting, fever, and abdominal pain. She was prepared for the operation following the clinical and radiological assessment. At laparotomy, appendicitis and left ovarian lesion torsion were found. An appendectomy and salpingo-oophorectomy were performed with no complications occurring during surgery or the follow-up period. The results of the histology analysis showed that the appendix was inflamed and that there was an epidermoid cyst with a wide infarction. CLINICAL DISCUSSION: Epidermoid cysts of the ovary are uncommon and often discovered by accident, and histological investigation is required for a definitive diagnosis. In our case, the signs and symptoms before surgery suggested appendicitis. Acute appendicitis in young children is also uncommon. Ovarian lesion torsion associated with acute appendicitis is extremely rare. The treatment is surgery and often requires appendectomy and salpingo-oopherectomy as in our case. CONCLUSION: Such cases should be considered in the differential diagnosis of abdominal pain at any age, and early diagnosis and surgery are always required.

Role of Magnetic Resonance Imaging (MRI) in grading gliomas comparable with pathology: A cross-sectional study from Syria.

Objective: Gliomas are one of the most common brain tumors in adults with a poor prognosis in most patients. Magnetic Resonance Imaging (MRI) plays a critical role in the diagnosis, management, and follow-up of gliomas. The aim of this study is to assess the sensitivity and specificity of MRI in the preoperative grading of supratentorial gliomas in comparison to histopathology. Methods: A cross-sectional study included 39 patients, aged between 40 and 75 years with histologically diagnosed supratentorial gliomas who underwent conventional MR imaging, which included T1, T2, and FLAIR sequences from November 2018-December 2019 in the Department of Neurosurgery, Tishreen University Hospital, Lattakia. The histopathological typing and grading of the tumor were done by using 2016 WHO classification. The sensitivity, specificity, predictive value, and accuracy of MRI in determining tumor grade were calculated. The comparison was done between MRI findings and WHO histopathological grading. Results: The overall sensitivity and specificity of MRI findings in the assessment of high-grade gliomas were 100% and 91% respectively. The positive predictive value (PPV) was 66.6%, and the negative predictive value (NPV) was 100%. The overall accuracy was 94.9%. The agreement between histopathological and MRI findings was 72%. Conclusions: MRI plays an essential role in the initial diagnosis and grading of supratentorial gliomas with high sensitivity and specificity. It is considered a non-invasive method and is useful in cases where the biopsy procedure is a contraindication or rejected by the patient.

Adamantinoma : The first report from Syria.

Adamantinoma is rare, representing less than 1 % of all primary bone tumors. It is a slow growing low grade tumor which is often clinically, radiologically and histologically mistaken for many other tumors. In this report, we present a case of Adamantinoma in a 17-year-old Syrian adolescent. The patient presented with pain and swelling anterior aspect of his left leg for last six months. We analyzed the clinical, radiographical and pathological characteristics of our patient, which helped us to reach the final diagnosis. The aim of this report is to document the presence of Adamantinoma in Syria to help other Syrian physicians considering this disease in the differential diagnosis if they face similar presentations.

Splenic cystic lymphangioma in adults: A rare case report from Syria.

Introduction and importance: Splenic lymphangiomas are an extremely rare entity that is mainly diagnosed in children. They are often found in the neck and axilla region. Cystic lymphangioma in the abdomen is unusual and the spleen is an exceptional location for lymphangiomas. Case presentation: We report a case of a 73 - year - old woman who presented with abdominal pain in the left upper quadrant for a month. Abdominal imaging studies show multiple splenic cysts. Splenectomy was performed and the specimen was sent for histopathologic examination. Microscopic inspection revealed splenic lymphangioma. Clinical discussion: Splenic lymphangiomas are uncommon lesions. Most cases have been reported in children. The head, neck, and axillary regions are the most common sites, whereas lymphangioma in the spleen is exceptional. Conclusion: Splenic lymphangiomas are uncommon benign tumors that are rarely seen during adulthood. Splenectomy is the preferred treatment.

Appendiceal neuroma presented as acute appendicitis: A rare case report from Syria.

INTRODUCTION AND IMPORTANCE: Appendiceal neuroma, also known as fibrous obliteration, is an exceptional benign lesion discovered in the vermiform appendix removed for indications of acute appendicitis. CASE PRESENTATION: We report a case of a 76-year-old woman who presented with signs and symptoms of acute appendicitis. An abdominal ultrasound revealed a mildly distended appendix with a peri-appendiceal fluid collection. Gross examination of the excised appendix showed an obliterated lumen of the distal part on serial cuts. Microscopically, the lumen is replaced by the proliferation of spindle cells into fascicles embedded in fibrous tissue. The diagnosis of appendiceal neuroma was reported and supported by the result of the S-100 immunohistochemical staining. CLINICAL DISCUSSION: An appendiceal neuroma represents an incidental finding in appendices excised for other reasons. Clinically, there are no specific signs or symptoms, and it is discovered inadvertently. Occasionally, it can mimic acute appendicitis. This lesion is characterized by the proliferation of spindle cells that occlude the lumen of the appendix. CONCLUSION: A histopathological evaluation is essential to achieve a definitive diagnosis of an appendiceal neuroma.

Metastatic melanoma to the gallbladder presented as a polyp with acute cholecystitis: A case report from Syria.

Introduction and importance: Malignant melanoma is one of the most aggressive unpredictable tumors that can metastasize to any organ. Metastases from cutaneous melanoma to the gallbladder are exceedingly rare. Most patients with gallbladder metastases from malignant melanoma are usually asymptomatic; therefore, its diagnosis can be a real challenge. Case presentation: We report a case of a 71-year-old woman with a past history of cutaneous melanoma, who clinically presented with signs and symptoms of acute cholecystitis. Ultrasound investigation of the gallbladder revealed intraluminal polyp. Gross inspection of the excised gallbladder showed a polypoid lesion in the fundus. Microscopically, the H&E-stained sections revealed nests of malignant cells, occasionally with pigmented cytoplasm. The diagnosis of metastatic melanoma was reported, and supported by the results of the immunohistochemical stains. Clinical discussion: Malignant melanoma is a very aggressive type of skin cancer. It arises from the melanocytes in the epidermis, uvea, meninges, and intestinal tract. Worldwide, only 40 cases of metastatic melanoma to the gallbladder were documented. Moreover, reviewing the English-language literature showed that primary melanoma is an extraordinary event and was described in about 28 cases. Clinically, metastases from cutaneous melanoma to the gallbladder are usually asymptomatic. Conclusion: We report a metastatic malignant melanoma to the gallbladder presented as a polypoid lesion and clinically manifested as acute cholecystitis.

Diagnostic challenge of a cystic solid pseudopapillary tumor in pancreas: A case report.

A solid pseudopapillary tumor should be included in the differential diagnosis of every pancreatic cystic lesion. A constellation of microscopic morphology and immunohistochemistry, in addition to the clinical history, aids in reaching the correct diagnosis.

An unusual case of chronic abdominal pain: an association between Celiac disease and Crohn's disease.

Inflammatory bowel diseases (IBD) and Celiac disease (CeD) are immune-mediated gastrointestinal diseases with incompletely understood etiology. Both diseases show a multifactorial origin with a complex interplay between genetic, environmental factors, and some components of the commensal microbiota. The coexistence of celiac disease with Crohn's disease is rarely reported in the literature. Here, we report a case of a 13-year-old Syrian male who presented with a history of abdominal pain, anorexia and pallor. CeD and Crohn's disease was documented on gastrointestinal endoscopy and histological study. The patient was treated with a gluten-free, low fiber, high caloric diet, and a course of oral corticosteroids with an improvement in growth rate and abdominal pain.

Primary splenic anaplastic variant of diffuse large B-cell lymphoma: a case report.

BACKGROUND: Primary splenic lymphoma represents a rare entity that constitutes less than 1% of non-Hodgkin lymphomas, and less than 2% of all lymphomas. Diffuse large B-cell lymphoma (DLBCL) is the most common histological subtype of primary splenic lymphomas. DLBCL encompasses a heterogeneous entity with distinct morphological variants. The anaplastic variant of DLBCL was first defined in the 2017 World Health Organization classification as a rare histological subtype that constitutes less than 3.4% of DLBCL cases. CASE PRESENTATION: A 65-year-old Syrian man presented to our hospital with constant dull localized left upper quadrant abdominal pain for about 20 days accompanied by general weakness, loss of appetite, and rapid weight loss. Clinical examination revealed isolated splenomegaly and left upper abdominal tenderness. Following physical, laboratory, and radiologic examinations, the patient underwent splenectomy. Interestingly, pathological and immunohistochemical examinations of the resected spleen confirmed the diagnosis of a primary anaplastic variant of DLBCL. CONCLUSIONS: Herein, we aimed to present an unusual combination of a rare splenic neoplasm and a unique lymphoma subtype. Furthermore, we aimed to highlight the difficulties in differential diagnosis and the importance of histological and immunohistochemical examinations with clinical correlation.

A large ovarian steroid cell tumor-not otherwise specified with a unique combination of benign and malignant features as a challenging cause of oligomenorrhea and hirsutism in a 21-year-old Syrian female: a case report.

BACKGROUND: Ovarian steroid cell tumors represent a rare category of sex cord-stromal tumors that constitute less than 0.1% of all ovarian tumors. These neoplasms are classified into three main subtypes according to the cell of origin: Leidyg cell tumors, stromal luteomas, and steroid cell tumors not otherwise specified (SCTs-NOS). The latter subtype is defined as a neoplasm of an uncertain lineage that mostly affects middle-aged women, whereas it's rare in younger ages. CASE PRESENTATION: We report a case of a 21-year-old virgin female who presented to our hospital with complaints of mild abdominal pain, hirsutism, and oligomenorrhea for more than a year. Before her current admission, the patient had attended an external gynecologic clinic where she had been prescribed oral contraceptives to regulate her periods. Nevertheless, on presentation to our institution, physical examination revealed abdominal tenderness with a palpable pelvic mass and mild hirsutism in the thigh. Ultrasonography demonstrated a large left ovarian mass measuring 154 × 104 mm, and compressing the uterus. Therefore, a unilateral salpingo-oophorectomy was performed, and interestingly, pathologic examination of the large aforementioned mass alongside with immunohistochemical correlation revealed the diagnosis of a large ovarian steroid cell tumor-not otherwise specified with a unique combination of benign and malignant features. CONCLUSIONS: Although ovarian steroid cell tumors represent a rare category, they must be considered in the differential diagnosis for mild virilization symptoms in young females due to the importance of early diagnosis and management. In this manuscript, we aimed to present the first case report from Syria that highlights the crucial role of detailed morphological examination for challenging cases despite the difficulties in differential diagnosis, and the absence of ancillary techniques. Furthermore, we managed to discuss a brief review of diagnostic methods, histological characteristics, and treatment recommendations.

Mesenteric inflammatory myofibroblastic tumor as a rare cause of small intestinal intussusception.

Inflammatory myofibroblastic tumor (IMT) is an uncommon, usually benign, mesenchymal tumor. IMT affects people of all ages, but it more commonly occurs in children and adolescents. Also, it has the potential to arise in any part of the body, though, it frequently develops in the lungs and mesentery. In this report, we discuss a rare clinical manifestation of mesenteric IMT presented as intussusception of the small intestine in a 7-year-old child.

Assessment of cervical intraepithelial neoplasia (CIN) with colposcopic biopsy and efficacy of loop electrosurgical excision procedure (LEEP).

PURPOSE: Conization for suspected high grade cervical intraepithelial neoplasia (CIN) is often performed based on abnormal cytology only. Loop electrosurgical excision procedure (LEEP) is a very common technique in this context. The present study analyses the accuracy of preoperative assessment of CIN with cytology plus colposcopic biopsy and assesses the efficacy of LEEP for the treatment of CIN. METHODS: Two-hundred and sixty-six consecutive patients treated with LEEP for suspected CIN at our center were retrospectively analyzed. Cytology, HPV-DNA testing, colposcopically directed cervical biopsy and/or endocervical curettage were performed to assess cervical lesions before and 3-6 months after surgery. RESULTS: Median age of the patients was 34 years. Median follow-up was 50 months. Preoperative HPV testing was positive for high risk types in 77.9%. All patients underwent LEEP without further ablative procedures. Complete excision of the lesion could be achieved in 84.3%; in 13.5% margins were not securely cleared and in 2.2% the lesion was not excised entirely. Overall complication rate was 5.4% (mainly postoperative bleeding and pain). Overall concordance of colposcopic biopsy and cone histology was 85.8%. The concordance rate was higher for CIN 2/3 (95.1%) compared with CIN 1 (63.2%). Nine patients (3.4%) had persistent disease after 3 months, 4 (1.5%) developed disease recurrence and underwent re-conization. HPV testing at 3-6 months after surgery was negative in 78.5%; 2 of the patients developing disease recurrence had a persistent HPV infection after surgery. CONCLUSIONS: Assessment of cervical lesions with colposcopic biopsy is an accurate method (concordance with cone histology 85.8%). Surgical treatment of high grade CIN with LEEP is a safe procedure with low recurrence rates, resulting in a clearance of cervical HPV infection in the majority of cases.

15-Hydroxyprostaglandin dehydrogenase associates with poor prognosis in breast cancer, induces epithelial-mesenchymal transition, and promotes cell migration in cultured breast cancer cells.

Breast cancer is the most frequent cancer and the leading cause of cancer-related deaths in women worldwide. The prognosis of breast cancer is tightly correlated with the degree of spread beyond the primary tumour. Arachidonic acid (AA) and prostaglandin E(2) (PGE(2)) are known to regulate tumour metastasis enabling epithelial-mesenchymal transition (EMT). However, the detailed role of 15-hydroxyprostaglandin dehydrogenase (HPGD), the key enzyme degrading prostaglandin E(2) , remains unclear in breast cancer. Here, we show that HPGD mRNA is overexpressed in a subset of clinical breast cancers compared to normal breast tissue samples and that high HPGD mRNA expression associates with poor prognosis. Immunohistochemical staining of primary breast cancer and lymph node metastasis tissue samples confirmed high HPGD protein expression in 20% of the samples, as well as associated HPGD expression with aggressive characteristics, such as increased risk of disease relapse and shorter disease-free survival. Results from cultured cells indicated abundant HPGD expression in highly metastatic breast cancer cells, and impairment of HPGD expression using RNA interference led to a significant decrease in transforming growth factor-ß signalling, in cellular arachidonic acid levels as well as in cell migration. Furthermore, gene expression microarray analysis followed by quantitative RT-PCR validation showed that HPGD silencing decreased aryl hydrocarbon receptor signalling and induced mesenchymal-epithelial transition. In conclusion, our results indicate that HPGD is highly expressed in metastatic and aggressive breast cancer and promotes EMT and migration in breast cancer cells.