Table 2 Summary of sites involved in EMD with presentation and treatment options.
Site involved | Presentation | Incidence | OS | Treatment options | Ref |
|---|---|---|---|---|---|
CNS- Brain parenchyma or meninges | Lethargy, nausea or vomiting, headache, confusion, paresthesia or seizures; visual, gait, and speech disturbances | 3% | 1 month | Whole brain radiation therapy, intrathecal chemotherapy, and systemic chemotherapy | [46] |
Skull | Smooth, firm, and non-tender mass on skull | <1% | High-dose dexamethasone | [3] | |
Orbit | Generally unilateral soft tissue orbital mass with complaints of headache, proptosis which is painless in nature, decreased vision, diplopia, restriction of eye movement and swelling, corneal crystalline deposits | <1% | 28 months | Local excision as a salvage surgery, whole brain radiation therapy, intrathecal chemotherapy, and systemic chemotherapy | [47] |
Vertebrae | Spinal cord or root compression, back pain | <1% | – | RT, intrathecal chemotherapy | [48] |
Breast | Breast lump ranging from 1 to 7.5 cms | 9% in primary EMD and 3% in secondary EMD | – | Surgical excision with adjuvant RT. Chemotherapy should be considered for tumors greater than 5 cm, high grade tumors and patients with refractory and / or relapsed disease. SCT. | |
Thyroid | Painful swelling on the side of the neck accompanied with odynophagia, dysphagia, and hoarseness | 2.9% | – | Chemotherapy with or without autologous SCT. External beam RT –when organ function loss is contemplated post-surgery. | |
Soft tissue of neck | Soft tissue swelling in the neck, unilateral nasal obstruction, more common in males, associated with epistaxis, facial swelling, pain and rhinorrhea. Can also present with headache, ptosis, diplopia, CN palsies II, III, IV, VI is sphenoid sinus is involved | 10% | – | Tumor size <5 cm-RT 30-40 Gy #20 Tumor size >5 cms- RT 40-50 Gy. Chemotherapy is considered if tumor size >5 cm, high-grade tumor, refractory/relapsed disease. Surgical excision may be considered. | |
Lungs | Unilateral Pleural effusion (right>left), pulmonary nodule, hilar mass, with atypical symptoms. Can have concurrent ascites | 2.65% | 2.8–4 months | Intrapleural bortezomib biweekly during induction and weekly or fortnightly during consolidation and maintenance along with systemic chemotherapy, concurrent pleurodesis or ICD drainage | [55] |
Spleen | Silent course, incidental finding on autopsies, can rarely present with left upper quadrant pain, painful splenomegaly, rarely splenic rupture | 9% in primary EMD and 11.5% IN secondary EMD | – | Splenectomy | |
Heart | Male preponderance, presents with dyspnea, tachycardia, pericardial effusion with or without tamponade, distant heart sounds, distended neck veins and positive kussmaul sign, pericardial or atrial mass | 0.4% | 13.5 weeks | Pericardial window for drainage, chemotherapy +/− high dose cortocosteroids | [57] |
Liver | Hepatomegaly, jaundice, ascites, and fulminant liver failure, mildly elevated liver transaminase levels | 28.8% | – | Systemic chemotherapy | [56] |
Pancreas | Decreased appetite, worsening peri-umbilical discomfort, pulsatile abdominal mass, bilateral rib pain, jaundice, homogeneous solid mass on CT | 2.3% | 7 months | Systemic chemotherapy such as with VRD, RT, SCTation | [58] |
Gatro-intestinal tract | Non-specific gastrointestinal symptoms, including anorexia and weight loss, abdominal pain, vomiting, and, rarely, gastrointestinal bleeding, usually from an ulcerated lesion. small bowel is the most common site of involvement, followed by stomach, colon, and esophagus. | <5% | – | RT or surgery along with systemic chemotherapy | [59] |
Omentum | Ascites, generally an autopsy finding | – | 1.5 months | – | [60] |
Testis | Testicular swelling, erythema, pain may or may not be present | 0.1% | Radical orchiectomy | [61] | |
Skin | Centrifugal appearance of multiple erythematous nodules or papules, or plaques that show a nodular or diffuse interstitial pattern. | 1.14% | 0.4–108 months (8.5 months) | Chemotherapy, RT, SCT | [62] |
Subcutaneous tissue | Single or multiple large highly vascularized subcutaneous nodules with a red-purple appearance | 0.6% | – | bortezomib-containing regimen followed by ASCT | |
Lymph node | Non-tender, enlarged lymph nodes. Weight loss maybe present. Most common site- paratracheal lymph node | 23.1% | – | – | [56] |
Muscle | Symmetric proximal muscle weakness and tenderness | 4.5% | – | Systemic chemotherapy | [11] |
Female reproductive system | Pelvic pain, profuse menorrhagia, and severe anemia | – | – | total abdominal hysterectomy with bilateral salpingo-oophorectomy | |
Adrenal glands | Incidental finding on imaging or autopsies | 7.7% | – | Surgical excision | [56] |
