Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia.

BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children occurring most commonly in the head and neck region. The treatment involves using a multimodality approach including chemotherapy, surgery, and radiation therapy. Survival for patients with localized disease has improved markedly, but the treatment of advanced disease remains a challenge. We report the clinical characteristics and outcome for patients treated at a tertiary care center in Saudi Arabia. METHODS: Patients aged 0-14 years diagnosed with RMS between 2005 and 2018 were included. Statistical analysis was performed using SPSS software. Kaplan-Meier method was used to calculate overall and event free survival. Cox proportional hazards model was used for multivariate analysis. RESULTS: One hundred and twenty-four patients were analyzed. The median age was 5.7 years with male predominance (2.4:1). The most common primary sites were head/neck (30%) and the genitourinary tract (25%). Embryonal RMS was present in 81%; alveolar in 19%. Most patients had intermediate risk disease (60%). The 5-year overall and event free survivals were 64.3% and 53.3%, respectively. Survival was influenced by primary tumor site, histology, and clinical risk group. Unfavorable primary site, high risk stratification, and poor initial response to therapy predicted a poor outcome. CONCLUSION: This study provides an insight on the current management outcomes for our patients with RMS. Cytogenetics and molecular diagnostics need to be incorporated as standard of care in the therapeutic approach of our patients. In addition, there is a need for national collaborative efforts to improve the outcome of RMS in children and adolescents.

Rosai-Dorfman Disease With Features of IgG4-Related Sclerosing Disease: A Case Report.

Rosai-Dorfman disease (RDD) is a rare condition characterized by the proliferation of non-Langerhans cell histiocytes that are associated with phagocytosed lymphocytes (emperipolesis). Clinically, it is classified into nodal, extra-nodal, neoplasia-associated RDD, and immune-related. Here, we present a case of a 65-year-old female who presented with facial pain following a dental procedure with no focal neurologic deficit. The MRI of the head and neck showed a well-defined lobulated soft tissue lesion with homogenous enhancement over the left cheek. Excision of the lesion was done, and the histopathological study reported extra-nodal RDD with features of IgG4-related sclerosing disease. The patient had no recurrence over the two years from the date of diagnosis.

Ovarian angiosarcoma: a case report and review of the literature.

INTRODUCTION: Sarcomas of the ovary can either be histologically pure or can represent components of a more complex tumor. Ovarian angiosarcomas are rare, and probably arise from carcinosarcomas, teratomas or the rich ovarian vasculature. To date, only two small case series have been published, one with four cases and the other with seven. CASE PRESENTATION: A 41-year-old Saudi woman presented to our gynecological clinic with abnormal vaginal bleeding. The initial clinical diagnosis was left ovarian cyst. The results of the remainder of her abdominopelvic examination were normal. Peri-operatively, the left ovarian mass resembled a hemorrhagic solid tumor. It was sent for frozen sectioning, which revealed it was an undifferentiated neoplasm. The final histopathological examination showed a vascular neoplasm showing vasoformative arborizing channels of variable sizes and shapes lined by atypical endothelial cells with intact capsule. Areas of necrosis were seen, along with fused anastomosing solid vascular area. She was diagnosed as having an angiosarcoma of intermediate grade, International Federation of Gynecology and Obstetrics stage IA. CONCLUSIONS: Patients with ovarian angiosarcomas most commonly present with abdominal pain, however some patients present with distant metastases, often in the lungs. Spread beyond the ovary is present at the time of diagnosis in most reported cases, with disease progression within less than a year after diagnosis. Cases of advanced stage disease behave aggressively and demonstrate poor response to surgery and chemotherapy, with an overall poor prognosis. They have a tendency for local recurrence and metastases, and prognosis is hence poor; the reported five-year survival rate is 10 percent to 35 percent, however, cases confined to the ovary have survived up to nine years.

Leiomyosarcoma metastatic to the thyroid diagnosed by fine needle aspiration cytology.

The thyroid gland is a known but an unusual site for metastatic tumours from various primary sites. Primary smooth muscle tumours of thyroid are rare. Leiomyosarcoma of the thyroid gland whether of primary or metastatic origin should be distinguished from anaplastic carcinoma. Few cases of leiomyosarcoma metastatic to thyroid, diagnosed by fine needle aspiration cytology (FNAC) have been documented. We are reporting this case to highlight the importance of FNAC as a tool for diagnosing sarcomas and to plan further management accordingly. Ancillary techniques can be applied on FNAC and give comparative results to tissue sections.

Dermatofibrosarcoma protuberance of the oral cavity: does it exist?

Dermatofibrosarcoma protuberance (DFSP) is a low grade spindle cell malignant tumour that is locally aggressive especially it incompletely excised. A 64-year-old man presented with intra-oral buccal mass of 34 year duration with accelerated increase in size in the last two years. CT scan showed well-circumscribed tumour with no relation to the overlying skin. Fine needle aspiration cytology revealed a highly cellular mitotically active spindle cell neoplasm with recommendation of excision with safety margins. Histological examination of the excised mass showed typical dermatofibrosarcoma protuberance with cytoplasmic positivity for Vimentin and CD34. Intra-oral sarcomas are rare and to the best of our knowledge only a single case of DFSP has been described in literature. We present another case for its unusual site, presentation, duration and surgical approach.

Gastrointestinal basidiobolomycosis: an unusual fungal infection mimicking colon cancer.

CONTEXT: -Basidiobolomycosis is a rare disease caused by the fungus Basidiobolus ranarum, an environmental saprophyte found worldwide. Patients with B ranarum infection may present with subcutaneous, gastrointestinal, or systemic lesions. Gastrointestinal basidiobolomycosis poses diagnostic difficulties, as its clinical presentation is nonspecific, with no identifiable risk factors. OBJECTIVE: -To discuss and compare the clinical features and histopathologic findings and other ancillary techniques that could be helpful in identifying gastrointestinal basidiobolomycosis. DESIGN: -We report 3 cases of gastrointestinal basidiobolomycosis and describe the clinical and morphologic findings while emphasizing the importance of identifying this unusual entity on endoscopic biopsies, thus avoiding unnecessary major surgeries. Fungal cultures were also performed, which are of diagnostic significance. Our first patient was lost to follow-up; however, patients 2 and 3 were followed up for 4 and 2 years, respectively. RESULTS: -In all 3 cases, patients presented with a clinical profile suggestive of malignancy. None of the patients gave any specific history. There was widespread abdominal disease with peritoneal involvement and colonic masses. Colonoscopic biopsy specimens showed nonspecific inflammation in 1 case; however, they showed only granulomatous inflammation in a second case and granulomas associated with fungal hyphae in a third. Typical morphology included hyphae, irregularly branched, thin-walled, occasionally septated and surrounded by a thick eosinophilic cuff (Splendore-Hoeppli phenomenon). CONCLUSION: -Gastrointestinal basidiobolomycosis can be detected on small endoscopic biopsy. The unequivocal diagnosis requires microbiologic cultivation of the fungus obtained from tissues. The prognosis for this disease is usually favorable as seen in 3 of our cases; however, cases with fatal outcome are on record.

Breast brucellosis in Taif, Saudi Arabia: cluster of six cases with emphasis on FNA evaluation.

BACKGROUND: Brucellosis is an endemic zoonosis seen in a variety of geographical locations such as the Middle East and Latin American and Mediterranean countries. Brucellosis is hyper-endemic in Saudi Arabia with an incidence of 5.4 per 1000 per year. Breast involvement very rarely occurs as a focal complication of brucellosis. METHODOLOGY: We report six cases of breast brucellosis retrieved from the files of the patients. Fine needle aspiration (FNA) was performed on all patients. In all cases direct smears were made at the patient's side for assessment of sample quality. Cytospin and cell block preparation was done and Gram, periodic acid-Schiff and Ziel-Nelson stains were done on all samples. Microbiological analysis included routine fungal and bacterial culture for tuberculosis. RESULTS: Age range was 20 to 48 years with a median of 22 years. Two patients gave history of raw milk ingestion, one was lactating, and one patient was pregnant. Cytomorphologic features of brucellosis in the breast include an abscess-like background with granulomatous inflammation. Cytology of the patients presented with breast abscess showed predominant neutrophilic infiltrate compatible with acute pyogenic inflammation. Culture was positive for B. melitensis in all cases. CONCLUSION: Brucellosis should be kept in mind in the differential diagnosis of breast abscess in endemic areas where ingestion of unpasteurized milk and milk products is common. Needle aspiration establishes a preliminary diagnosis and has the advantage of material submission for microbiologic cultures and provides focal treatment along with long-term combined antibacterial treatment for the control of systemic infection.