Clinical features and prognosis of multiple myeloma and orbital extramedullary disease: Seven cases report and review of literature

Table 1 Baseline clinical characteristics

Items	Data
Age (yr)	41.4
Gender: M/F (cases)	3/4
Type of MM: IgGk/k (cases, %)	5 (71.4)/2 (28.6)
Stage of ISS: I/II/III (cases)	3 (42.9)/2 (28.6)/2 (28.6)
High-risk cytogenetics in FISH: 1q21+ (cases, %)	2 (28.6)
Features of EMD (cases)	2 (28.6)
Spinal cord/thyroid/breast/armpit soft tissue/ovary/pleural effusion/brain	2/2/2/2/2/1/1/0
≥ 2 sites (cases, %)	6 (85.7)
≥ 5 cm diameter (cases, %)	2 (28.6)
PC (%)	2.5
Calcium > 2.75 (mmol/L, C)	0
Creatinine > 176.8 μmol/L (mmol/L, R) (cases, %)	1 (14.3)
Hemoglobin < 100 (g/L, A) (cases, %)	1 (14.3)
Bone lesion (B) (cases, %)	7 (100)
Albumin < 35 g/L (cases, %)	0
β2-MG ≥ 5.5 mg/L (cases, %)	1 (14.3)
LDH > 240 U/L (cases, %)	1 (14.3)

M: Male; F: Female; MM: Multiple myeloma; ISS: International Staging System; IgG: Immunoglobulin G; k: k light chain; FISH: Fluorescence in situ hybridization; EMD: Extramedullary disease; PC: Plasma cell % in bone marrow; β 2-MG: β 2-microglobulin; LDH: Lactatedehydrogenase.

Table 2 Ocular clinical characteristics

Case	Gender/age (yr)	Chief complains	Ocular symptoms	Sites of orbital Lesions and Size	Operation treatment	Pathological diagnosis	Extraocular involvement
1	Male/44	Enlarged left cheekbone mass for 8 mo	Left eyelid drooping, blurred vision three months later	Left eyelid drooping, blurred vision	Endoscopic tumor resection via nasal sella approach in March 2019	Pasmacytoma of sellar region and clivus, infiltrating bone and submucosal	Left cheekbone mass 3 cm × 3 cm and mid-sternal mass 6 cm × 4 cm
2	Female/41	Left eye socket swollen, lightly proptosis for more than one year	Left eye socket swollen, slightly proptosis and/or eye movement disorders	Left tear gland, mass size was unknown	The thyroid mass and left orbital lacrimal gland mass in May 2018 and April 2019	left lacrimal gland plasmacytoma	Skull bone destruction and thyroid mass
3	Male/54	Diagnosed with MM because of lung infection and elevated globulin in September 2011	Blurred vision and proptosis and/or eye movement disorders in the right eye 2 yr later	The right orbital muscle and the left medial and lateral rectus muscles in orbital CT, mass size was unknown	No	No	Skull, ribs, lumbar skin, and mediastinum. The right ninth rib and L5 paravertebral multiple soft tissues, the largest mass reached 4.9 cm × 4.4 cm.
4	Male/42	Diagnosed with MM because of bone pain in December 2007	Blurred vision in left eye more than 3 yr later	Retina in left eye by head and eye MRI, mass in left retro-ocular choroid, size was 0.3 cm × 0.2 cm	Topical laser treatment	No	Cervical, thoracic and sacral vertebral bone lesion
5	Female/39	Pain in the arch of the eyebrow and blurred vision in February 2010	Pain in the arch of the eyebrow and blurred vision	left orbit mass size was 2.8 cm × 2.6 cm in PET-CT	Left orbital mass, bilateral uterine appendage and left breast mass excision in February and September 2010, respectively	Left orbital plasmacytoma	Bone lesion of thoracic vertebra ,Left breast, and ovarian mass, diagnosed with MM 8 mo later, left breast mass size was 12 cm × 10 cm in chest CT
6	Female/37	Diagnosed with MM because of bone pain and increased creatinine in 2011	Blurred vision in both eyes more than 4 yr later	Optic canal lesions in both eyes by head and eye MRI, the soft tissue mass were 1.5 cm × 0.8 cm and 0.9 cm × 0.3 cm in the right and left part	No	No	Sacral wing, sphenoid, clivus bone and pituitary
7	Female/33	Diagnosed with MM because of Bone Pain and Lumbar Mass Biopsy in July 2014	Discomfort and proptosis and/or eye movement disorders in the right eye more than 4 yr later	Right orbital inner and outer wall and musculoskeletal space mass, the right orbital mass was about 1.5 cm in diameter	Lumbar fracture internal fixation and tumor resection, right orbital tumor resection in July 2014 and March 2019, respectively	Right orbital plasmacytoma	Multiple bone destruction throughout the body

CT: Computed tomography; MRI: Magnetic resonance imaging; MM: Multiple myeloma; PET: Positron emission tomography.

Table 3 Treatment options and response

Case	Regimens	Treatment options	Response
1	VRD, ECHOP and lenalidomide maintenance therapy	Chemotherapy after surgery	PR
2	BAD and lenalidomide maintenance therapy	Chemotherapy and radiotherapy after surgery	PD soon after CR, then reached PR after undergoing Car-T therapy in another hospital
3	T-CAD, BAD and thalidomide maintenance therapy	Chemotherapy	CR
4	T-CAD and thalidomide maintenance therapy	Chemotherapy after surgery	CR
5	T-CAD, BAD, B-CEVAD and thalidomide maintenance therapy	Chemotherapy and radiotherapy after surgery	CR
6	T-CAD ,BAD and thalidomide maintenance therapy	Chemotherapy	CR
7	CAD,R-DECP, BED and lenalidomide maintenance therapy	Chemotherapy after surgery	CR

VRD: Bortezomib, Lenalidomide and Dexamethsone; BAD regimen (Bortezomib, Doxorubicin and Dexamethsone); CAD: Ifosfamide, Epirubicin or Piraubicin and Dexamethsone; T-CAD: Thalidomide and CAD; B-CEVAD: Bortezomib, Etoposide, ifosfamide, Vindesine sulfate, Epirubicin, and Dexamethsone; BED regimen: (Bortezomib, Etoposide and Dexamethsone; CR: Complete remission; PR: Partial remission; PD: Disease progression.