A 6-year-old child complains of bilateral thigh pain, motor weakness, and some sensory deficits of both lower limbs; he has a history of bladder dysfunction over the past 2 weeks; MRI of the spinal cord shows a mass arising from the filum terminale and conus medullaris causing some pressure effect. A CNS tumor is suspected. Which of the following tumor is MOST likely arising in such site?

Correct Answer: C

Rationale: The correct answer is C) myxopapillary ependymoma. In this scenario, the child presents with symptoms suggestive of a tumor in the filum terminale and conus medullaris region, which is characteristic of myxopapillary ependymoma. This tumor arises from the ependymal cells lining the spinal cord and is commonly found in the lower spinal cord region. Option A) medulloblastoma is a malignant brain tumor commonly found in the cerebellum, not in the spinal cord. Option B) anaplastic astrocytoma is a high-grade glioma that typically arises in the brain, not in the spinal cord. Option D) choroid plexus carcinoma is a rare tumor arising from the choroid plexus in the brain, not in the spinal cord. Educationally, understanding the specific characteristics and locations of different CNS tumors is crucial for accurate diagnosis and management in pediatric nursing practice. Recognizing the typical presentations of various tumors helps healthcare providers make informed decisions regarding treatment and care for pediatric patients with CNS tumors.

Correct Answer: E

Rationale: The correct answer is E because it is the one that does not accurately describe renal cell carcinoma (RCC) in children. In pediatric cases, RCC is not associated with von Hippel-Lindau disease, which is more commonly seen in adults. Option A is correct as patients with RCC in children can present with symptoms like hematuria, flank pain, and a palpable mass. Option B is also accurate since RCC can be asymptomatic and incidentally detected on imaging studies. Option C is true as RCC in children has a propensity to metastasize to the lungs, bone, liver, and brain. Educationally, understanding the presentation and characteristics of RCC in children is crucial for pediatric nurses to provide timely and appropriate care. Recognizing the differences between RCC in children and adults, such as the absence of von Hippel-Lindau disease association, helps in accurate diagnosis and management. This knowledge ensures comprehensive care and support for pediatric patients with RCC.

Correct Answer: B

Rationale: In pediatric osteosarcoma, the worst prognostic factor among the options given is poor histologic response to treatment (Option B). This is because a poor response to treatment indicates that the tumor is more aggressive and less likely to be effectively controlled or eradicated. A good histologic response is associated with better outcomes, including higher rates of survival and lower risk of recurrence. Option A (primary pelvic bone tumor) is not as poor a prognostic factor as poor histologic response because the primary tumor's location alone does not necessarily dictate the response to treatment or overall prognosis. Osteosarcoma can occur in various bones, and while pelvic tumors may present challenges due to the proximity to vital structures, they are not inherently associated with worse outcomes. Option C (bony metastases at the time of diagnosis) is also a serious factor but not as detrimental as a poor histologic response. Metastases indicate disease spread, which can complicate treatment, but response to therapy still plays a significant role in determining outcomes. Option D (lung metastases at the time of diagnosis) is a negative prognostic factor but is not as critical as poor histologic response. Lung metastases are common in osteosarcoma and can impact prognosis, but the response to treatment remains a crucial determinant of overall success. Educationally, understanding prognostic factors in pediatric osteosarcoma is vital for nurses caring for pediatric oncology patients. Recognizing the significance of histologic response can guide nursing assessments, interventions, and patient education efforts. Nurses play a key role in monitoring treatment responses, managing side effects, and supporting patients and families through the challenges of osteosarcoma treatment.

Correct Answer: D

Rationale: In Langerhans cell histiocytosis (LCH), the organ that is LEAST likely affected is the middle nodes, making option D the correct answer. LCH is a rare disease characterized by the overproduction of histiocytes, which are a type of white blood cell. LCH commonly affects the skin, bones, and other organs but rarely involves the lymph nodes, particularly the middle nodes. Option A, the skin, is often affected in LCH, presenting as skin rashes or lesions. Option B, the ear, can also be affected in LCH, leading to symptoms like hearing loss or ear discharge. Option C, bones, are commonly involved in LCH, causing bone pain or fractures due to the infiltration of histiocytes. Understanding the typical organ involvement in LCH is crucial for pediatric nurses as they care for children with this condition. Recognizing the less common sites of involvement, like the lymph nodes, helps in early detection and appropriate management. By knowing the disease manifestations, nurses can provide holistic care and support to pediatric patients and their families affected by LCH.

Correct Answer: B

Rationale: The correct answer is B) neurofibromatosis. Neurofibromatosis, specifically type 1 (NF1), is a genetic syndrome associated with an increased risk of optic glioma, a type of tumor that occurs along the optic nerve. This condition commonly presents in childhood and can lead to vision problems if left untreated. Option A) Down syndrome is a genetic disorder caused by the presence of an extra chromosome 21. While individuals with Down syndrome may have an increased risk of certain health conditions, optic glioma is not typically associated with this syndrome. Option C) Monosomy refers to a genetic condition where a cell has only one copy of a particular chromosome instead of the usual two. This is not specifically linked to an increased risk of optic glioma. Option D) Bloom's syndrome is a rare genetic disorder characterized by short stature, sun sensitivity, and an increased risk of cancer. While some individuals with Bloom's syndrome may develop tumors, optic glioma is not a common feature of this condition. Educational Context: Pediatric nursing practice requires a thorough understanding of various genetic syndromes and their associated health risks. Recognizing the signs and symptoms of conditions like neurofibromatosis is crucial for early detection and intervention to prevent potential complications such as optic glioma. By understanding the distinctive features of different genetic syndromes, nurses can provide comprehensive care and support to pediatric patients and their families.