Paraspinal Desmoid Tumor in a Pediatric Patient with No Surgical History: A Case Report.

Desmoid tumors are locally aggressive, benign neoplasms originating in connective tissues. Although the exact pathophysiology remains unknown, antecedent trauma or surgery are believed to be important contributing factors. The occurrence of paraspinal desmoid tumor in pediatric patients is extremely uncommon.

Morphologic and Ancillary Studies of Pediatric Acinic Cell Carcinoma: A Single Institute Experience.

Background: Acinic cell carcinoma (AciCC) is the second most common pediatric malignant salivary gland tumor. However, there are limited pathology publications about this tumor in the pediatric population.

Methods: We describe four pediatric AciCC cases diagnosed between 2000 and 2021 in our institute.

Morphologic Spectrum of Rhabdomyomatous Mesenchymal Hamartomas (Striated Muscle Hamartomas) in Pediatric Dermatopathology.

Background: Rhabdomyomatous mesenchymal hamartomas (RMHs), also termed striated muscle hamartomas, are rare benign tumors of skin and subcutis, which mostly occur at birth with a predilection for the head and neck. Simple surgical excision is the treatment modality of choice with excellent prognosis.

Objective: To review the spectrum of the different clinical and pathologic features of RMHs in pediatric patients and recognize their characteristics to avoid confusion with other lesions in their list of differential diagnosis.

Prognostic variables in patients with primary soft tissue sarcoma of the extremity and trunk treated with neoadjuvant radiotherapy or neoadjuvant sequential chemoradiotherapy.

Background: Neoadjuvant radiotherapy (NRT) is an effective strategy to treat soft tissue sarcomas (STS). However, the role of neoadjuvant chemoradiotherapy (NCRT) remains to be determined.

Methods: From May 1999 to July 2010, 112 patients with localized STS of the extremity and trunk who were treated with NRT or NCRT followed by surgery were retrospectively reviewed.

Multimodality management of metastatic patients with soft tissue sarcomas may prolong survival.

Objectives: Patients who develop metastatic disease from soft tissue sarcoma have a poor prognosis. The purpose of this study was to identify metastatic survival rates and identify prognostic variables that predict for these outcomes.

Methods: Between 2000 and 2010, 182 patients with stage I to IV primary soft tissue sarcomas of the extremity and trunk were treated with multimodality treatment.

Malignant gastrointestinal neuroectodermal tumor: clinicopathologic, immunohistochemical, ultrastructural, and molecular analysis of 16 cases with a reappraisal of clear cell sarcoma-like tumors of the gastrointestinal tract.

The clinical, histologic, immunophenotypic, ultrastructural, and molecular features of a distinctive gastrointestinal tumor are described. Sixteen patients, 8 women and 8 men aged 17 to 77 years (mean age, 42 y; 63% less than 40 y) presented with abdominal pain, intestinal obstruction, and an abdominal mass. Mean tumor size was 5.

Unusual pulmonary findings in mucolipidosis II.

We report undescribed pulmonary findings in a child with mucolipidosis II (ML-II). Children with ML-II bear significant pulmonary morbidity that may include extensive pulmonary fibrosis, persistent hemosiderosis as well as pulmonary airway excrescences as they reach preschool age.

Congenital panfollicular nevus associated with polydactyly.

A 2-year-old girl presented with ulnar-sided duplication of the left thumb distal to the interphalangeal joint and syndactyly of the first web space. She also had several asymptomatic pink-tan cutaneous papules, involving the first and second ray of the left hand and wrist, clinically resembling a linear epidermal nevus. Microscopically, the papules were composed of well-circumscribed aggregates of basaloid epithelium within the dermis.