ABSTRACT
BACKGROUND: Chordomas are rare, slow-growing, locally aggressive, malignant tumors of the spine. Chordomas are conventionally treated with surgical resection with or without radiation. There is an absence of literature documenting the natural history of a primary sacral chordoma. CASE DESCRIPTION: A 65-year-old man presented with rectal pain, constipation, urinary and fecal incontinence, S1 radiculopathy, and a palpable rectal mass. A needle biopsy confirmed the pathologic diagnosis of sacral chordoma. The patient declined to have surgery because of the surgical risks involved. He was managed conservatively with supportive care only. The patient was routinely followed in clinic and had a subjective and objective excellent quality of life with adequate pain management. Meanwhile, his neurologic status did not deteriorate. During follow-up, some posterolateral aspects of the chordoma regressed. However, the bulk of the lesion continued to slowly progress. The patient survived for 7.5 years. He eventually succumbed to urosepsis and new-onset peritoneal metastasis. CONCLUSIONS: To our knowledge, the patient is the only documented case in the literature of an untreated biopsy-proven sacral chordoma. The patient's tumor was intended for resection, and therefore comparable with data from treated chordomas. The patient's survival is similar to the median survival in treated chordomas. The patient's survival was despite negative prognosticators, such as advanced age of the patient and high sacral location above S2.
Subject(s)
Chordoma/physiopathology , Conservative Treatment , Sacrum , Spinal Neoplasms/physiopathology , Treatment Refusal , Aged , Chordoma/diagnostic imaging , Disease Progression , Humans , Magnetic Resonance Imaging , Male , Pain Management , Spinal Neoplasms/diagnostic imaging , Spinal Neoplasms/pathology , Tomography, X-Ray ComputedABSTRACT
Phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMT-MCT) are tumors that may cause tumor-induced osteomalacia and rarely appear intracranially. The authors describe the case of an 8-year-old girl who was found to have PMT-MCT with involvement of the cerebellar hemisphere and a small tumor pedicle breaching the dura mater and involving the skull. This was removed surgically in gross-total fashion without further complication. Histologically the tumor was confirmed to be a PMT-MCT. There was no evidence of tumor-induced osteomalacia. At the 42-month follow-up, the patient is doing well, has no abnormalities, and is free of recurrence. PMT-MCTs are rare tumors that may involve the brain parenchyma. A gross-total resection may be effective to cure these lesions.
Subject(s)
Ataxia/etiology , Biomarkers, Tumor/analysis , Cerebellar Neoplasms/surgery , Fibroblast Growth Factors/analysis , Hypophosphatemia, Familial/etiology , Mesenchymoma/surgery , Skull Neoplasms/surgery , Biomarkers, Tumor/genetics , Cerebellar Neoplasms/chemistry , Cerebellar Neoplasms/complications , Cerebellar Neoplasms/pathology , Child , Diagnosis, Differential , Female , Fibroblast Growth Factor-23 , Fibroblast Growth Factors/genetics , Gene Expression Regulation, Neoplastic , Humans , Hypophosphatemia/etiology , Magnetic Resonance Imaging , Mesenchymoma/chemistry , Mesenchymoma/complications , Mesenchymoma/pathology , Osteomalacia/etiology , Reverse Transcriptase Polymerase Chain Reaction , Skull Neoplasms/chemistry , Skull Neoplasms/complications , Skull Neoplasms/pathology , Tomography, X-Ray Computed , Treatment OutcomeABSTRACT
Primary gastrointestinal (GI) lymphomas constitute 5%-10% of all gastrointestinal tumors. Involvement of the entire GI tract as multiple polypoid lesions is very rare. Multiple lymphomatous polyposis is a distinctive and rare type of malignant GI lymphoma. Multiple lymphomatous polyposis is thought to represent mantle cell lymphoma of the GI tract. Approximately 60 cases of MLP have been reported so far. We report the first case of diffuse large B-cell lymphoma presenting as multiple lymphomatous polyposis of the GI tract in a 49-year-old patient with HIV and describe the clinicopathologic features. The patient was treated with a combination therapy of highly active antiretroviral therapy and CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) and exhibited complete remission.
