Malignant Dendritic Cell Sarcomas in the Skin: 2 Cases of Rare Sarcoma Subtypes With Literature Review.

ABSTRACT: Interdigitating dendritic cell sarcoma is a rare, aggressive hematological malignancy primarily originating in lymph nodes, with only 10 reported cases presenting in the skin (primary cutaneous interdigitating dendritic cell sarcoma). Past presentations showed erythematous nodules on the proximal extremities, back, or face. Morphologically, these neoplasms are similar to melanomas and other dendritic cell (DC) tumors, making their diagnosis difficult. Here, we present 1 case of primary cutaneous interdigitating dendritic cell sarcomas and another 1 of malignant indeterminate dendritic cell tumor (indeterminate DC sarcoma). The first case is an 83-year-old man who presented with recent ulceration and bleeding of an asymptomatic, slow growing lesion on his right thigh with biopsy revealing a large, well-circumscribed polypoid spindle cell tumor in the dermis with atypical cells with vesicular nuclei in a lymphoplasmacytic background and immunohistochemistry positivity for CD45, CD68, S100, and Cyclin D1. The second case is a 74-year-old man who presented with a progressively darkening and enlarging abdominal skin lesion with biopsy revealing a diffuse infiltrate of atypical poorly differentiated pleomorphic nuclear cells and immunohistochemistry positivity for S100, CD1a, CD56, CD43, cyclin D1, CD31, CD4, and BRAF V600E. Our findings contribute to expand the reported literature on primary cutaneous DC sarcomas.

An Unexpected Response of Orbital Retinoblastoma to Inconsistent Systemic Chemotherapy Alone.

Introduction: A 16-month-old girl presented with bilateral germline retinoblastoma, with advanced retinoblastoma in the right eye and severe orbital retinoblastoma in the left eye. Patients typically receive treatment with the multimodal protocol consisting of chemotherapy, surgery, and radiotherapy, which has improved survival. Case Presentation: Our patient elected for medical management with systemic chemotherapy instead of enucleation or exenteration. She received three blocks systemic chemotherapy consisting of vincristine sulfate, etoposide phosphate, and carboplatin, with irregular intervals between blocks over 3 years, due to poor compliance. Following chemotherapy, she had tumor regression in both eyes and no signs of active disease 13 years later. Conclusion: This case represents an interesting favorable outcome of orbital retinoblastoma management with systemic chemotherapy alone despite inconsistency versus conventional multimodal protocol.

Dogs Licks Are Not Benign: Pasturella Multocida Bacteremia From Household Dog.

Pasturella (P.) multocida is a gram-negative coccobacilli commonly colonized in the oral, nasopharyngeal, and upper respiratory tracts of animals. Infections due to P. multocida range in severity, and symptoms largely depend on underlying immune status and co-morbid conditions. Widely known, the transmission of P. multocida is commonly thought to occur through biting and skin breakage alone. However, multiple studies have highlighted instances of severe complications secondary to transmission through the passage of P. multocida through animal licking alone without skin disruption. Here, we present a case of a nonagenarian female presenting with septic shock secondary to P. multocida with the source of transmission found to be secondary to the patient's dog licking her chronic leg wounds. We also highlight other instances of similar transmission through a literature review, including common treatment courses. We aim to raise awareness of common transmissions of bacteria, specifically P. multocida, along with broadening differentials when one presents with skin and soft tissue infections.

Ophthalmic manifestations of Lamb-Shaffer syndrome: a case presentation and literature review.

Lamb-Shaffer syndrome, caused by haploinsufficiency of SOX5, leads to a unique constellation of dysmorphic features and intellectual delay. The SOX5 family of proteins plays an integral role in neuronal development. We present the clinical traits of an 11-year-old boy with Lamb-Shaffer syndrome and highlight the ocular findings of the syndrome reported thus far in the literature. Approximately 55% of all patients reviewed had some form of ocular abnormality associated with Lamb-Shaffer syndrome, including, predominantly, strabismus as well as optic nerve abnormalities, epicanthal folds, and refractive errors, highlighting the potential significance of SOX5 on neurologic development.