Embryonal tumor with multilayered rosettes: Post-treatment maturation and implications for future therapy.

BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR) is a deadly grade IV pediatric brain tumor. Despite an intensive multimodal treatment approach that includes surgical resection, high-dose chemotherapy, and radiotherapy, the progression-free survival at 5 years is less than 30%. CASE: We report a case of long-term survival in a 5-month old female with a large mass in the posterior fossa, diagnosed as ETMR, which subsequently underwent treatment-induced maturation. Prior to chemotherapy, histopathology revealed an abundance of highly proliferative, undifferentiated cells and multilayered rosette structures. Conversely, post-treatment histopathology revealed cell populations that differentiated into neuronal and ganglionic phenotypes. At 5-year follow-up, the patient remains progression-free. CONCLUSION: This finding contributes to the few reports to date of post-treatment differentiation/maturation of ETMR cell populations, with an implication for less cytotoxic therapeutic interventions aimed at differentiation.

Primary Cutaneous Clear Cell Sarcoma, Clinical Outcome With Sentinel Lymph Nodes Status.

OBJECTIVES: Primary cutaneous clear cell sarcoma (PCS) is a rare malignancy and difficult to differentiate from melanoma. We investigated factors influencing survival and recurrence. METHODS: An institutional cancer registry and literature search were used for a retrospective study. Only clear cell sarcoma cases with a primary site of skin and subcutaneous tissue were included. Kaplan-Meier and Cox regression analyses were used to assess survival time and hazard ratios. RESULTS: Three eligible cases were identified at our institution. In addition, the PubMed and Google Scholar reviews identified 1,878 items, with 23 patients with PCS. The median age was 25 years with 62% female. The tumors ranged in size from 0.4 to 4.5 cm. Cytogenetics showed t(12;22)(q13;q12) in all cases and a unique variant of t(2;22)(q32.3;q12) in one case. Surgery was the most common treatment, followed by chemotherapy/radiation. PCS recurred in 46% of patients with a median relapse-free survival time of 15 months. Only two known PCS-related mortalities were recorded, at 38 and 60 months following initial diagnosis. Smaller tumor size and negative sentinel lymph node biopsy (SLNB) status were significantly associated with a better disease-free survival. CONCLUSIONS: Tumor size and SLNB status influence PCS survival and recurrence. More research is needed due to the rarity of this disease.

Massive malignant solitary fibrous tumor arising from the bladder serosa: a case report.

INTRODUCTION: Solitary fibrous tumors are rare neoplasms of mesenchymal origin. They are often of low malignant potential and rarely metastasize. While they frequently arise from the pleura, they can occur at any soft tissue site in the body. We present a case of a large (28 × 21 cm) malignant solitary fibrous tumor arising from the bladder serosa. In addition, the clinicopathologic features, differential diagnosis, cytogenetics and management of this rare disease are discussed, along with a review of the existing literature on this topic. CASE PRESENTATION: An otherwise healthy 41-year-old Caucasian man presented with weight loss and progressive abdominal bloating. A subsequent computed tomography scan of his chest, abdomen and pelvis revealed a 26.8 × 21 cm intra-abdominal mass occupying most of his abdominal cavity. The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder. He underwent operative resection and the resected mass measured 28 × 21 × 18 cm and weighed 4.8 kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern, with randomly assorted tan fleshy nodules. A histologic evaluation revealed variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma. In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity. The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder. Our patient is currently eight months post-surgery without evidence of recurrence. CONCLUSIONS: Extrapleural occurrences of solitary fibrosis tumors are being increasingly observed. Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare. As there are no pathognomonic features of malignancy, surgical resection is often both diagnostic and therapeutic, as was the case in our report.

Osteomyelitis of the patella caused by Legionella anisa.

A 51-year-old man with a history of stage IV angioimmunoblastic T-cell lymphoma was diagnosed with osteomyelitis of the patella. Legionella anisa was identified by 16S rRNA gene sequencing and culture. The patient had pneumonia 2 months prior to this osteomyelitis episode. L. anisa was retrospectively detected in his lung tissue by 16S rRNA gene sequencing and was considered the source of the L. anisa that caused his patella osteomyelitis.