Primitive neuroectodermal tumors (PNETs) are a heterogeneous group of malignant neoplasms found primarily in childhood and early adulthood. In this paper, we described the case of a 64-year-old male with primary spinal PNET, successfully treated with surgery, craniospinal radiotherapy, and concurrent chemotherapy. This is the case of a 64-year-old male who presented with a 2-month history of bilateral lower extremity weakness and numbness associated with urinary and bowel incontinence. Work-up was done, and the spine's plain magnetic resonance imaging (MRI) revealed a heterogeneously enhancing intradural lesion with an extradural component at the right T9/T10 level, causing mild to moderate cord compression. The patient underwent laminectomy and gross total resection of the said tumor. Histology and immunohistochemistry were consistent with a primitive neuroectodermal tumor of the spine. The tumor recurred three months after the surgery, and the patient was then referred for radiation therapy with concurrent chemotherapy. Repeat spinal MRIs with three- to six-month intervals after treatment showed no tumor recurrence as of August 2021. Primary spinal PNETs are rarely found in adults, especially in the elderly. These tumors currently have no recommendations or guidelines regarding their management. Thus, most cases are presently being managed based on studies on children and central nervous system (CNS) PNETs. This paper presented a case of a successfully treated primary spinal PNET in the elderly. The management was primarily based on studies done on that of the pediatric population and CNS PNETs.
Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral

Introduction: Ependymomas are slow-growing neuroectodermal tumors that may arise from various parts of the central nervous system. Anaplastic ependymoma represents 3-5% of ependymomas, and it is rarely found in adults and the infratentorial area, particularly the cerebellum. This paper discusses the first reported case of an adult female with anaplastic cerebellar ependymoma who underwent surgery and was treated with chemotherapy for tonsillar herniation.

Case Presentation: This is a case of a 58-year-old Filipino female with a five-month history of dizziness, headache, nausea, and vomiting. Cranial computed tomography (CT) scan revealed the presence of hydrocephalus with enhancing lesions at the right cerebellum. The patient underwent ventriculoperitoneal shunting (VPS) with sub-total excision and biopsy of the right cerebellum. Histology and immunochemistry were consistent with a high-grade anaplastic ependymoma (WHO Grade III). Cerebrospinal fluid and spinal magnetic resonance imaging (MRI) were negative for tumors. The initial plan was to undergo limited field external beam radiation therapy to the cerebellum. However, the patient was lost to follow-up. Two months after surgery, she presented with similar symptoms. MRI revealed tonsillar herniation and interval progression of the mass compressing the fourth ventricle, pons, and medulla oblongata; thus, medical decompression urgent chemotherapy with cisplatin and etoposide were started. After four chemotherapy sessions, repeat cranial MRI revealed resolution of tonsillar herniation and interval regression of the mass.

Conclusion: This paper presented a rare case of anaplastic cerebellar ependymoma with tonsillar herniation, successfully treated with chemotherapy. Radiotherapy is the standard of care following surgical resection. Still, our case management showed that in a patient with tumor progression presenting with tonsillar herniation, alternative management is to give systemic chemotherapy instead of radiotherapy.

Introduction: Lung cancer is the leading malignancy metastatic to the central nervous system with approximately 20% to 44% of all cases developing brain metastasis. Immunotherapy using pembrolizumab, an anti-PD1monoclonal antibody, is a novel method in lung cancer treatment and has shown favorable results in patients with metastatic brain lesions from non-small cell lung carcinoma (NSCLC). However, the cost of the recommended treatment dose limits its use especially in developing countries like the Philippines. Case Presentation: The authors report two patients with lung cancer with brain metastasis upon diagnosis. The first patient is a 65-year-old male, non-smoker with PD-L1 expression of 60%. He was started on pembrolizumab 100 mg IV every three (3) weeks and a repeat CT scan after 11 cycles revealed a reduction of the two brain metastatic lesions and no fluoro-D-glucose (FDG) uptake on positron emission tomography (PET) scan even after one year into treatment. The second patient is a 67-year-old female, a previous smoker with PD-L1 expression of 50% with a metastatic solitary solid nodule in the cortex of the right cerebellum. After five cycles of pembrolizumab 100 mg IV every three weeks, there was noted complete resolution of brain metastasis on PET scan even after one year of treatment. Conclusion A lower dose of pembrolizumab (100 mg given every 3 weeks) was found to be effective in the management of advanced NSCLC with brain metastasis in the two patients. Further studies are recommended to investigate lower dose pembrolizumab as monotherapy without radiation therapy or surgery in patients with NSCLC with brain metastasis especially in the setting of a resource-limited country like the Philippines.
Immunotherapy ; Lung Neoplasms ; Developing Countries

Background: Olfactory neuroblastoma, also known as esthesioneuroblastoma, is a rare malignant tumor that presents as a locally aggressive disease. It accounts for an incidence of 0.4 per million population affecting men and women equally in all ages. As olfactory neuroblastoma is rare, having an intracranial extension is even more unusual. Only a few cases have been reported in literature, hence, there is no widely accepted standard of care. Case Presentation: This is a case of a 33-year-old female who presented with a 4-month history of nasal congestion which progressed to a rapidly growing nasal mass and bilateral proptosis. She soon became bedridden and exhibited signs of increased intracranial pressure. Imaging revealed a heterogeneously enhancing nasal cavity mass with intracranial extension. Biopsy and immunohistochemistry confirmed the olfactory neuroblastoma. She subsequently underwent chemotherapy and radiotherapy since the tumor was unresectable. In a span of three months, after only three cycles of chemotherapy with cisplatin and etoposide and thirty fractions of radiation therapy, the patient significantly improved from being completely bedridden to an ambulatory individual capable of self-care. We completed eight cycles of chemotherapy and referred to surgical specialists for possible resectability. However, the patient did not consent for surgery and opted to have palliative care. Conclusion Most cases of olfactory neuroblastoma are treated through combination therapy. The patient significantly improved from the administration of chemotherapy with cisplatin and etoposide plus radiotherapy. This case report shows the significance of chemotherapy with radiotherapy as the treatment of choice for late-stage olfactory neuroblastoma in which surgery is not amenable.
Esthesioneuroblastoma, Olfactory ; Drug Therapy ; Radiotherapy

Background: Dermatomyositis - a rare autoimmune myositis – is a disease affecting primarily the skin and muscles which has been correlated with an elevated risk of solid tumors - commonly affecting the ovaries, breast, colon and nasopharynx. However, there is a rare association between dermatomyositis and pulmonary large cell neuroendocrine carcinoma such that in a thorough literature review of published material, only two cases have been reported internationally and none locally. Large cell neuroendocrine carcinoma - in itself, is also a rare malignancy representing only 1-3% of all primary lung carcinomas. Case Presentation: This is a case of a 53-year-old Filipino female, hypertensive, diabetic, dyslipidemic, hypothyroid - nonsmoker – who presented with an eight-month history of facial erythema, swelling of bilateral metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints, and erythema over extensor surfaces of the MCP and PIP joints. She had markedly elevated creatine kinase MM and positive anti-nuclear antibody for which she was prescribed prednisone, which she did not comply with. She lost weight and experienced severe abdominal pain. Abdominal imaging subsequently revealed multiple confluent abdominal and thoracic lymphadenopathy with histopathology of large cell neuroendocrine carcinoma (LCNEC). Peculiar to this case however is that despite being a lung carcinoma, the scan showed no pulmonary masses or nodules. Immunohistochemical stains of the lymph node were positive for neuroendocrine markers: pancytokeratin, synaptophysin, TTF-1 and negative for any mutation in the epidermal growth factor receptor. Her Ki-67, which is used as a prognostic factor and correlates with mitotic count - was 70% and PD-L1 tumor proportion score – a predictor of therapeutic effect - is 5-10%. She was subsequently diagnosed with dermatomyositis and pulmonary LCNEC. She has presently completed her 8th cycle of cisplatin and etoposide and has gained weight. Presently, her musculocutaneous lesions have resolved. However, a repeat PET scan was done still showing multiple confluent paraaortic, aortocaval, pericaval lymph nodes with no significant interval change from the first PET scan. Next generation sequencing had been requested showing DIS3 to be the gene altercation – however, as of this writing, no available therapeutic modalities are available to target this. Patient was nonetheless given Pembrolizumab for 3 cycles and subsequently expired due to complications of pneumonia. Conclusion Among published data, we herein present the third reported case of dermatomyositis associated with pulmonary large cell neuroendocrine carcinoma worldwide and the first reported case in the Philippines thereby contributing to the present medical literature. This case demonstrates two rare diseases associated with each other and exemplifies the need for an awareness of such disease entities. It demonstrates a rare case of LCNEC peculiarly without any pulmonary masses or nodules. It also illustrates the necessity in evaluating patients with dermatomyositis for their respective risk in terms of malignancy and other immunocompromised states. Lastly, it contributes to the knowledge on therapeutic options that may be given to patients presenting with both disease entities.
Dermatomyositis ; Lung Neoplasms

This is a rare case of primary pachydermoperiostosis (PDP). A 28-year-old Filipino male presented with a lifelong history of enlarged hands and feet. He eventually developed symmetrical swelling of the ankles and knees associated with leg heaviness and knee pain with difficulty with ambulation, hence consult. His eldest brother also had the same “elephant-like” extremities. He had cutis vertices gyrata with a thickened corrugated hair pattern, deep lines on the forehead, deepened nasolabial folds, enlarged extremities especially distally, with coarse, thick skin, and prominent clubbing. The nails were convex “watch crystal-like.” The wrists, knees, and ankles were tender and enlarged, with massive effusion of the knees. All joints were devoid of warmth and erythema. Skeletal survey favored hypertrophic osteoarthropathy over acromegaly, with periosteal thickening of the metaphysis and digital clubbing. The filarial smear was negative for blood parasites. Skin biopsy showed keratoderma. Synovial fluid was non-inflammatory while arthroscopic synovial biopsy showed chronic inflammation eosinophilic amorphous tissue. Electrocardiogram, echocardiogram, thyroid function tests, complete blood count, peripheral blood smear, serum chemistries, coagulation tests, urinalysis, urine electrolytes, fecalysis, and chest CT scan were unremarkable. Whole abdomen ultrasound revealed the liver parenchymal disease. Hepatitis profile revealed chronic infection with hepatitis B, with low infectivity. The three major criteria for PDP (pachydermia, periostitis, and digital clubbing) were fulfilled. Possible secondary causes were either excluded or were non-contributory. He was started on analgesics and anti-inflammatory medicines. Repeated arthrocenteses drained liters of synovial fluid per knee, and along with intra-articular steroid injections and compressive bandages, temporarily relieved his bilateral knee pain. He was referred to rehabilitation to maximize his range of motion and to address body image issues. The patient remains on regular follow-up for periodic arthrocentesis. The option of anti-VEGF treatment and arthrotomy was explored as possibilities but were not deemed practical. PDP is a rare genodermatosis. Life span is not affected but the quality of life is dismal without supportive management, as there is no known cure. A multidisciplinary team composed of a rheumatologist, dermatologist, orthopedic surgeon, plastic surgeon, rehabilitation physician, and a psychiatrist should be available to assist in the needs of these patients.
Osteoarthropathy, Primary Hypertrophic