Posts

Rhabdomyosarcoma (RMS) of the orbit

Rhabdomyosarcoma “A child with sudden, rapidly progressing unilateral proptosis with an orbital mass → think Rhabdomyosarcoma until proven otherwise.” Malignant tumour of mesenchymal origin showing skeletal muscle differentiation.  Most common primary orbital malignancy in children . Epidemiology Accounts for ~4% of all paediatric cancers . 10% of all RMS cases occur in the orbit . Age: usually 5–7 years (rare after adolescence). Sex: slight male predominance . Sites of Origin Orbit (most common intraocular adnexal site). Can arise from extraocular muscles, connective tissue, or blood vessel walls . Histological Subtypes Embryonal (60–70%) – most common in orbit; relatively better prognosis. Alveolar (20–25%) – more aggressive, seen in older children/adolescents. Pleomorphic (rare) – in adults, very aggressive. Botryoid variant – polypoid form under mucosal surfaces. Clinical Features R...

Glioblastoma Multiforme

Glioblastoma multiforme (GBM) is the most aggressive and most common primary malignant brain tumour in adults. It is classified as a WHO Grade 4 astrocytoma and is notorious for its rapid progression, resistance to therapy, and poor prognosis. Pathophysiology Origin: Arises from astrocytes , a type of glial cell in the central nervous system. Genetics: Primary GBM (de novo): Often seen in older adults; associated with EGFR amplification , PTEN mutations , and chromosome 10 loss . Secondary GBM (progresses from lower-grade gliomas): Seen in younger patients; associated with IDH1/2 mutations and TP53 mutations . Features: High cellularity with pleomorphic, hyperchromatic nuclei Pseudopalisading necrosis Microvascular proliferation (glomeruloid bodies) Diffuse infiltration beyond visible margins.   Clinical Features Symptoms depend on location but may include: Headache ...

Iris Melanoma

Iris malignant melanoma is the most common primary neoplasm of the iris and can be most easily seen by the physician on clinical examination. It is usually a small, discrete lesion, although it may occasionally be diffuse and infiltrative; multiple; and result in heterochromia, glaucoma, chronic uveitis, or spontaneous hyphema. Many patients are often aware of a pigmented spot on the iris that has been present for many years and only recently has begun to grow. With a slit lamp, gonioscopy, and serial slit lamp photography, the ophthalmologist can document the size and growth of the tumor without biopsy. Fluorescein angiography may be helpful in demonstrating the vascularity of the lesion, but is not diagnostic. Ultrasonography is of little value in the evaluation of iris melanoma. Iris melanoma is relatively benign with a 5-year survival rate of approximately 95%. This is related to the fact that iris melanomas are predominantly of the spindle A-cell type and are usually smaller...

Cancer of the Colon

General Information Cancer of the colon is a highly treatable and often curable disease when localized to the bowel. Surgery is the primary form of treatment and results in cure in approximately 50% of patients. Recurrence following surgery is a major problem and often is the ultimate cause of death. The prognosis of colon cancer is clearly related to the degree of penetration of the tumor through the bowel wall and the presence or absence of nodal involvement. These 2 characteristics form the basis for all staging systems developed for this disease. Bowel obstruction and bowel perforation are indicators of poor prognosis. Elevated pretreatment serum levels of carcinoembryonic antigen (CEA) have a negative prognostic significance. Many other prognostic markers have been evaluated retrospectively in the prognosis of patients with colon cancer, although most, including allelic loss of chromosome 18q or thymidylate synthase expression, have not been prospectively validated. Microsatell...

Malignant Mesothelioma

Cellular Classification Histologically, mesotheliomas are composed of fibrous or epithelial elements or both. The epithelial form occasionally causes confusion with peripheral anaplastic lung carcinomas or metastatic carcinomas. Attempts at diagnosis by cytology or needle biopsy of the pleura are often unsuccessful. It can be especially difficult to differentiate mesothelioma from adenocarcinoma on small tissue specimens. Thoracoscopy can be valuable in obtaining adequate tissue specimens for diagnostic purposes. Examination of the gross tumor at surgery and use of special stains or electron microscopy can often help. The special stains reported to be most useful include periodic acid-Schiff diastase, hyaluronic acid, mucicarmine, CEA, and Leu M1. Histologic appearance appears to be of prognostic value, with most clinical studies showing that epithelial mesotheliomas have a better prognosis than sarcomatous or mixed histology mesotheliomas. Stage Information Patients with sta...

Adult Acute Lymphoblastic Leukemia in Remission

Current approaches to postremission therapy for adult acute lymphoblastic leukemia (ALL) include short-term, relatively intensive chemotherapy followed by longer-term therapy at lower doses (maintenance), high-dose marrow-ablative chemotherapy or chemoradiotherapy with allogeneic stem cell rescue (alloBMT), and high-dose therapy with autologous stem cell rescue (autoBMT). Several trials of aggressive postremission chemotherapy for adult ALL now confirm a long-term disease-free survival rate of approximately 40%. In the latter 2 series, especially good prognoses were found for patients with T-cell lineage ALL, with disease-free survival rates of 50% to 70% for patients receiving postremission therapy. These series represent a significant improvement in disease-free survival rates over previous, less intensive chemotherapeutic approaches. In contrast, poor cure rates were demonstrated in patients with Philadelphia chromosome-positive (Ph+) ALL, B-cell lineage ALL with an L3 phenotype (...

Untreated Adult Acute Lymphoblastic Leukemia

Standard treatment options for remission induction therapy: Most current induction regimens for adult acute lymphoblastic leukemia (ALL) include prednisone, vincristine, and an anthracycline. Some regimens also add other drugs, such as asparaginase or cyclophosphamide. Current multiagent induction regimens result in complete response rates that range from 60% to 90%. Two subtypes of adult ALL require special consideration. B-cell ALL which expresses surface immunoglobulin and cytogenetic abnormalities such as t(8;14), t(2;8), and t(8;22)] is not usually cured with typical ALL regimens. Aggressive brief duration high-intensity regimens similar to those used in aggressive non-Hodgkin's lymphoma have shown high response rates and cure rates (75% complete remission; 40% failure-free survival). T-cell ALL, including lymphoblastic lymphoma, similarly has shown high cure rates when treated with cyclophosphamide-containing regimens. Whenever possible, such patients should be en...