RESUMEN
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is an extremely rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL) that typically presents in middle-aged patients and carries a poor prognosis. Hypercalcemia presenting as the initial manifestation of the disease is rare, with only one other case reported in the literature. We report a case of a 90-year-old male who presented with progressive lethargy and unintentional weight loss. Initial workup showed elevated serum calcium of 14.6 mg/dL, corrected for albumin, and creatinine of 1.51 mg/dL. He had a suppressed iPTH of 6.3 pg/mL and normal PTHrP (13 pg/mL). Computed tomography (CT) scan of the abdomen and pelvis was performed to rule out underlying malignancy, which showed splenomegaly and enlarged retrocrural and porta hepatis lymph nodes. Bone marrow biopsy was performed to evaluate for hematological malignancy, which revealed findings diagnostic of THRLBCL. While rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) is one of the mainstay therapies for DLBCL and has been shown to have comparable outcomes in THRLBCL, there are documented concerns with its toxicity profile limiting the ability of older patients (60 years and older) to complete therapy. Our patient was treated with R-mini-CHOP, which is much better tolerated in this patient demographic. R-mini-CHOP features decreased doses of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with the conventional dose of rituximab. This case discusses a rare subtype of non-Hodgkin lymphoma presenting with a unique manifestation of hypercalcemia. We highlight the importance of thorough investigation for causes of hypercalcemia as well as the efficacy and tolerability of R-mini-CHOP in this elderly patient demographic.
RESUMEN
Primary aortic sarcoma is a rare and aggressive malignancy with only approximately 190 cases reported in the literature. While angiosarcoma and intimal sarcomas represent an estimated 67.7% of malignant aortic tumours, spindle cell sarcomas are even more exclusive, consisting of only 0.9% of malignant aortic tumours. Differentiated from other malignant aortic tumours, spindle cell sarcomas are of mesenchymal origin and usually express vimentin and osteopontin. Clinical presentations are variable and nonspecific, ranging from back pain, abdominal pain or elevated blood pressure, misleading to differentials like pulmonary emboli or aortic aneurysms such as in our case here. In this article, we discuss the finding of an extremely rare aortic sarcoma masquerading as a pulmonary embolism. The patient underwent surgical resection; however, the course was complicated by the development of brain metastases and intracranial haemorrhage. The literature is expanding regarding the evolution of adjuvant chemotherapy and radiation therapy in the treatment of these patients. The exact pathogenesis of spindle cell sarcomas is unknown but thought to be related to the MDM2-p53 pathway. The development of spindle cell sarcomas may be related to Li-Fraumeni syndrome, which should be on the differential for these patients. This case highlights the importance of identifying aortic sarcomas in patients who present with signs and symptoms of peripheral embolization as the diagnosis can be easily misconstrued for thrombus or aortic aneurysm, leading to a delay in proper and timely management. We herein emphasize that aortic sarcomas should be included in the clinician's working differential due to the poor prognosis and outcomes that these aggressive tumours carry. LEARNING POINTS: Malignant aortic tumours are rare and can present with a multitude of symptoms ranging from constitutional symptoms to abdominal discomfort to unexplained hypertension. Spindle cell sarcomas represent 1 of the least common malignant aortic tumours reported in the literature.Malignant aortic tumours have a poor prognosis, and of the various types of malignant aortic tumours, aortic sarcomas have a particularly poor prognosis with a 5-year survival rate of 8%.The exact pathophysiology of these malignancies is unknown but is thought to be related to the MDM2-p53 pathway and may be related to Li-Fraumeni syndrome.
RESUMEN
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is an extremely rare morphologic subtype of diffuse large B-cell lymphoma (DLBCL), accounting for only 1-3% of total cases. It is considered an aggressive lymphoma with a poor prognosis. Hypercalcemia has been described as an uncommon presenting symptom of patients with DLBCL in several case reports. Here, we report an unusual case of severe hypercalcemia in a patient who was ultimately diagnosed with T-cell/histiocyte-rich B-cell lymphoma. A 69-year-old male patient presented to our hospital with nausea, vomiting, weakness and unintentional weight loss. His initial blood tests showed a serum calcium level of 16.1 mg/dL and serum creatinine level of 3.25 mg/dL. He had high intact parathyroid hormone (PTH, 6.8 pg/mL), mildly elevated 25-hydroxyvitamin D and serum PTH-related peptide (PTHrP). To exclude malignancy, computed tomography (CT) scans of the chest, abdomen and pelvis were performed which were unremarkable. A bone marrow biopsy was performed to detect any hidden hematologic malignancy which showed large mononuclear cells with prominent nucleoli and occasional Reed-Sternberg cells, consistent with the diagnosis of THRLBCL. Subsequent positron emission tomography demonstrated diffuse fluorodeoxyglucose (FDG) uptake. This case reports a unique presentation of a rare subtype of non-Hodgkin's lymphoma. We highlight the importance of pursuing a thorough workup for causes of hypercalcemia as well as understanding the underlying mechanisms of severe hypercalcemia in malignancy.
