plasmacytomas by godfrey thuku msiv. outline case presentation case presentation types of plasma...
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PlasmacytomasPlasmacytomas
By Godfrey Thuku MSIVBy Godfrey Thuku MSIV
OutlineOutline
Case PresentationCase Presentation Types of plasma disordersTypes of plasma disorders Radiosurgery treatment Radiosurgery treatment
Case presentationCase presentation
42yr old Hispanic female with a slowly growing right scalp cyst since 42yr old Hispanic female with a slowly growing right scalp cyst since May 2009May 2009
Symptoms of left arm and fingers tingling, headaches. No systemic Symptoms of left arm and fingers tingling, headaches. No systemic signssigns
Cyst was approx 5cm in circumference, no erythema, tenderness to Cyst was approx 5cm in circumference, no erythema, tenderness to palpation, no discharge palpation, no discharge
No history of Trauma to the site No history of Trauma to the site
PMH;PMH; Hypertension and hypercholesterolemia. Hypertension and hypercholesterolemia. PSH;PSH; 2 cesarean 2 cesarean sections. sections. FMH;FMH; Hypertension, stroke, CAD, hypercholestrolemia. Hypertension, stroke, CAD, hypercholestrolemia. SH;SH; Does not smoke or drink. Does not smoke or drink. PE; PE; 2 inch diameter lesion on the right 2 inch diameter lesion on the right scalp. Rest of exam intactscalp. Rest of exam intact
Neuro;Neuro; Completely neurologically intact. Completely neurologically intact.
11stst OR visit OR visit
Aborted excision of scalp cystAborted excision of scalp cyst
During dissection an irregular During dissection an irregular contour was noted as well as contour was noted as well as calcified areas.calcified areas.
High suspicion lesion was not High suspicion lesion was not a cyst or lipoma.a cyst or lipoma.
Obtained skull x-rays which Obtained skull x-rays which demonstrated a clear defect demonstrated a clear defect in the skull.in the skull.
Right frontal craniectomyRight frontal craniectomy
Stereotatic surgical planningStereotatic surgical planning
Resection of tumor attached Resection of tumor attached to durato dura
Dura resectionDura resection
Dural reconstructionDural reconstruction
22ndnd OR visit OR visit
Plasma cell disordersPlasma cell disorders
Plasma cell granulomasPlasma cell granulomas – monoclonal expansion of – monoclonal expansion of single plasma cell. Its an inflammatory processsingle plasma cell. Its an inflammatory process
Plasmacytoma – Plasmacytoma – a discrete solitary mass of neoplastic a discrete solitary mass of neoplastic monoclonal plasma cells within in either bone or tissuemonoclonal plasma cells within in either bone or tissue
Extramedullary plasmacytomasExtramedullary plasmacytomas – localized cell – localized cell neoplasms arising within soft tissuesneoplasms arising within soft tissues
Solitary bone plasmactyomaSolitary bone plasmactyoma – solitary lytic bone lesion – solitary lytic bone lesion
Multiple myelomaMultiple myeloma – rarely curable systemic malignancy – rarely curable systemic malignancy of plasma cells.of plasma cells.
Clinical featuresClinical features
Multiple MyelomaMultiple Myeloma Median age – 60-75Median age – 60-75 Gender – slight male predominanceGender – slight male predominance Bone pain precipitated by movement Bone pain precipitated by movement High levels of Monoclonal proteinHigh levels of Monoclonal protein
Pathology; especially to Pathology; especially to rule out rule out plasmagranulomaplasmagranuloma
Lab tests; CBC, Lab tests; CBC, Biochemical screen Biochemical screen including electrolytes and including electrolytes and corrected sodium, serum corrected sodium, serum and urine proteins, and urine proteins, immunoglobulin levels, immunoglobulin levels, full skeletal surveyfull skeletal survey
TreatmentTreatmentRadiotherapy – treatment of choiceRadiotherapy – treatment of choiceRecommended dose – 40gy in 20 fractions for SBP of 5cm or less.Recommended dose – 40gy in 20 fractions for SBP of 5cm or less.
63yr old man with Hx of successful stem cell rescue with 63yr old man with Hx of successful stem cell rescue with vertex headaches and hoarseness.vertex headaches and hoarseness.
MRI showed a mass in left clivus extending to foramen MRI showed a mass in left clivus extending to foramen magnum.magnum.
Tumor was unresponsive to dexamethasone for six monthsTumor was unresponsive to dexamethasone for six months
Decision made to treat it as a plasmacytomaDecision made to treat it as a plasmacytoma
Treated with fractioned cyberknife, 400 cGy for five daysTreated with fractioned cyberknife, 400 cGy for five days
ProsPros No screw-induced No screw-induced
numbnessnumbness Rare cases of pin Rare cases of pin
site cellulitis with site cellulitis with AVM’sAVM’s
Potential infectionsPotential infections
ConsCons Can be Can be
compromised by compromised by patient motion esp patient motion esp around the skull around the skull basebase
No histologic confirmation of No histologic confirmation of diagnosisdiagnosis
Pt had complete radiographic response within three monthsPt had complete radiographic response within three months
Serial head MRI for 12+ months without neurological Serial head MRI for 12+ months without neurological toxicitytoxicity
70yr old female with Hx of infiltrative ductal 70yr old female with Hx of infiltrative ductal carcinoma had developed cavernous sinus carcinoma had developed cavernous sinus syndrome (headache, left side numbness and syndrome (headache, left side numbness and earache, double vision) earache, double vision)
Has a solitary mass in left carvenous sinusHas a solitary mass in left carvenous sinus Probable metastases from breast, surgery done Probable metastases from breast, surgery done
but pathology revealed a plasmacytoma. but pathology revealed a plasmacytoma. Systemic evaluation showed MMSystemic evaluation showed MM
Pt underwent gamma knife radiosurgery followed Pt underwent gamma knife radiosurgery followed by chemo.by chemo.
ProsPros Tumor responded Tumor responded
very rapidly to very rapidly to radiationradiation
ConsCons Clinical symptoms Clinical symptoms
never resolvednever resolved Lack of proper Lack of proper
diagnosis work-updiagnosis work-up
ConclusionsConclusions
Proper diagnostic work-up, including Proper diagnostic work-up, including systemic labssystemic labs
Literature indicates plasmacytomas Literature indicates plasmacytomas are very radiosensitive.are very radiosensitive.
Would not hesitate to use Would not hesitate to use radiosugeryradiosugery