CPC

Dr K Kishore Chandra
2nd yr DM resident
Medical oncology
 History
• A 21 year old male, resident of Medchal, presented with
weakness of both lower limbs for 4 years, parasthesias in
both feet for past 3 months and loss of weight since 1 year.

• He was apparently alright around 5 years ago, when he

sustained injury to right hip and lower back due to slip and
fall in his house.

• He had pain in the right hip and lower back for few days,
which subsided.
• Almost a year later, he had low back ache, involving the lower
lumbar region, midline, increasing on prolonged sitting.

• It was relieved on walking around, no radiation of pain to lower

limbs, no radicular symptoms, no typical EMS or buttock pain.

• History of right hip pain over right iliac crest for 4 years which is
on and off, increasing on prolonged sitting.
• There was difficulty in squatting and getting up from squatting position for 4
years. This progressed to difficulty in climbing stairs for past 1 year with further
progression in the past 3 months.

• He is able to walk on level ground without support and able to get up from high
rise chair with minimal support.

• No history of any slippage of slippers

• He gives history of stiffness in upper limb while doing over head activities and is
unable to complete the range of motion.

• No h/o neck weakness/difficulty in getting up from supine position/turning to

sides in bed
• History of stiffness of both lower limbs for 3
years, increased when trying to squat or fold
his legs, with difficulty in flexing knees
completely.

• History of parasthesias and numbness on

both plantar surface of feet over toes and
distal part of forefoot for 3 months.
• He also gave history of a swelling over right infra
scapular and infra axillary region since 3 years, which
was gradually increasing in size, slightly painful.

• There was no redness or warmth over swelling, not

associated with fever.

• He complained of diffuse hyperpigmentation of face

and body for 1 year, more over soles and palms, no
pigmentation over buccal mucosa
• There was h/o loss of appetite and significant weight loss (from 70
kg to 55 kg).

• History of SOB on exertion for 1 year, on climbing stairs. Now since 3

months, unable to climb stairs [both due to sob and weakness], no
history of SOB at rest.

• No history of cough /chest pain /palpitations

• History of both leg swellings for past 7 days upto ankles, now slightly
resolved after foot end elevation, no facial puffiness/abdominal
distention/decreased urine output.
• There was history of nocturia- 3 times/night for past 3
months, no history of frothuria.

• History of joint pains involving both knee joints and right

ankle, increased on changing posture from sitting to
upright ,no typical EMS or swelling of joints.

• History of myalgias,more over thighs than arms.

• No history of loss of sensations.

• No history of bladder/bowel movement.

• No history of cranial nerve involvement.

• No history of altered sensorium , memory

deficits, headache, seizures.
• No h/o fever, cough, chest pain or palpitations.

• No history of vomiting /abdominal pain /loose

stools/abdominal distention/giddiness.

• No history of skin rashes, alopecia/oral

ulcers/photosensitivity/sicca
symptoms/RP/sclerodactyly
Past H/o: No history of PTB in past. He had COVID-19
illness [mild] in the form of loss of taste and smell-3
months back, was home isolated and took no
treatment.

• The patient had multiple admissions to other hospitals

in the past and had been treated conservatively.

Family history: No history of CTD/arthritis in family.

 
Examination details:
• On examination, pt is conscious, cooperative, afebrile,
Pedal edema present [mild pitting type R > L]

• PR- 90/min, BP-130/80mmHg, RR- 20/min, SPO2- 98% on RA

• Height: 5 feet 8 inches weight: 59 kgs BMI- 19.9

• LN – Bilateral cervical LN present, multiple small discrete,

soft, non tender and largest measuring 3X2 cm. Axillary LN
present- largest measuring 3X2 cm in right axilla- central
group
• Wasting of buccal fat present and temporal fat +, no wrist sign /thumb
sign/high arched palate

• CVS- S1S2 present, no murmurs

• RS- Right lateral chest wall- over posterior aspect- near infrascapular and
infraxillary area- swelling of around 12 X 12 cm with ill defined margins,
variable in consistency, soft- firm, mild tenderness +, no warmth or
erythema over swelling+.

• B/L NVBS, no crepts.

• P/A- soft, splenomegaly: 2 cm below LCM. Hepatomegaly: 3 cm below RCM

• CNS- HMF and cranial nerves-Normal
Power Neck 2/5
• Motor-
BULK : Wasting of both gluteal muscles+
SIDE RIGHT LEFT
BULK : THIGHS 42 40.5
CALVES 32 32
TONE UL N N
LL N N
• POWER UL– Weakness of serratus anterior (difficulty in overhead abduction),
rhomboids and subscapularis+
SHOULDER 4+/5 4+/5
ELBOW 4+/5 4+/5
WRIST 5/5 5
HAND GRIP 100 % 100%
TRUNK N
•LL
Hip Flexors, abductors 3+/5 3+/5
Adductors
Extension 3-/5 3-/5
Knee flexion 4-/5 4-/5
Extension 4-/5 4-/5
Ankle 5/5 5/5
•DEEP TENDON REFLEXES B T S K A
R - - - -
L - - - -
•SENSORY: Fine touch/crude touch/pin prick N
Joint position sense impaired at bilateral 1st MTP
Vibration sense lost at both malleoli
•RHOMBERG -negative
MSK: TJ : right shoulder, both knees and ankles
SJ : Right ankle
• ROM: RIGHT HIP FLEXION: terminal part restricted
IR AND ER: NORMAL
ABDUCTION AND ADDUCTION: normal
KNEE: TERMINAL PAINLESS RESTRICTION OF FLEXION
• No deformities
• SPINE: Lower lumbar spine mild tenderness present, no deformity

• Forward flexion restricted due to stiffness in posterior aspect of

thigh

• Lateral flexion: restricted

No SI joint tenderness

• Bilateral Patrick’s negative 

• The patient was thoroughly investigated

Clinical images
Clinical images
Test Results day 1 Results day 10
Hb 15.5gm/dl 15.9gm/dl
Hct 45% 47.8%
RBC count 5.43 mil/cumm 5.84 mil/cumm
MCV 82.8fl 84.5fl
MCH 28.5 pg 28.2 pg
MCHC 34.4gm/dl 33.4gm/dl
RDW - SD 37.6fl 39.8 fl
TLC 10300/cumm 10200/cumm
DLC N 78,L15,M5,E2 N57,L33,M7,E2
Platelet count 4.5L/cumm 4.8L/cumm
PS Comment RBCs are NN, TC N with
neutrophilia, Platelets are
adequate
ESR 3mm/1st hr 2mm/1st hr
Urine Ph 5.5, SG 1.015,
PR/KET/SUR – NEG, Micro-
NAD
HBSAg, HCV, HIV Negative
SGOT 13 U/L
SGPT 8 U/L
ALP 65 U/L
TP 6.9 gm/dl
Alb 3.8 gm/dl
PT T-13.4 sec, C – 12, INR –
1.14
APTT T – 33.9, C – 32 sec
Urea 19 mg/dl
Creatinine 0.78 mg/dl
CPK 52 U/L
T3 0.94 1.04 1.1-3.1nmol/L
T4 8.86 9.7 4.5-11.7ug/dl
TSH 8.56 10.3 0.2-4 uIU/ml
Ca 8.5 mg/dl
Phos 4.58 mg/dl
Vit D total 120.47 ng/ml
Procal 0.35 ng/ml
FSH 7.96 mIU/ml 1.5-12
LH 9.86 mIU/ml 1.7-8.6
Cortisol 13.19mcg/dl 10-20mcg/dl
Kappa FLC 53.85 mg/L 3.3-19.4
Lambda FLC 78.3 mg/L 5.7-26.3
FLC ratio 0.67 0.26 – 1.65
Beta2 microglobulin 3.5mg/L
ANA 4+
C ANCA Negative
P ANCA Negative
Anti dsDNA Negative
C3 137
C4 46
MRI LS Spine 2017 Small posterocentral disc protrusion of L5-S1
disc with mild thecal sac indentation
X ray chest 2019 Ill defined widening with irregular margin
along posterolateral aspect of right 9th rib
CT Chest 2019 Minimal right pleural effusion with pleural
thickening and expansile osteolytic lesion of
right 9th rib. Possible fibrous dysplasia
MRI Chest with contrast 2019 Monostotic fibrous dysplasia posterior part of
right 9th rib
MRI whole spine Jan 2021 Minimal scoliosis of dorsal spine and mild
bulges of L3-L4, L4-L5, L5-S1 compressing
thecal sac with spinal canal narrowing
MRI Pelvis with SI Joint Jan 2021 B/L SI joint normal, Mild diffuse STIR
hyperintensities in SC planes in pelvic
muscles involving B/L gluteal muscles,
ileopsoas,obturator internus and
externus,pectineus & adductors/ likely
inflammation S/O myositis
PET CT Feb 2021 Metabolically active expansile lytic lesion
in right 9th rib with infiltration of adjacent
chest wall, muscles unlikely to be fibrous
dysplasia
Non FTG avid cervical and axillary LN
ENMG summary S/O Demyelinating motor sensory
neuropathy with muscle disease
2D Echo No RWMA, Good B/L Ventricular function.
No MR/TR/AR/PAH. No PE/Veg/clots
IMAGING
STUDIES
Summarizing Imaging findings
• Solitary, expansile ,lytic ,FDG avid right rib lesion
with minimal soft tissue component

• B/L cervical , axillary, inguinal lymph nodes with

minimal FDG Uptake
• Right pleural effusion
• Splenomegaly
LYTIC RIB LESION DDs

• Fibrous dysplasia
• Lymphoma
• Plasmacytoma
• Ewing’s Sarcoma
• Histiocytosis (Eosinophilic Granuloma )
• Infective etiology (tubercular)
• Chondroid neoplasms
• Metastasis
Fibrous Dysplasia

For Against
• Age • Cortical break
• Site • Soft tissue component
• FDG Uptake • Pleural effusion
• FDG uptake
Lymphoma

For Against
• Splenomegaly • Site
• Symmetrical • Expansile lesion
lymphadenopathy
• Soft tissue component
• FDG Uptake
Plasmacytoma

For Against
• Age
• Expansile lytic lesion
• Soft tissue component
• FDG Uptake
Ewing’s sarcoma of Rib
For Against
• Age • Small soft tissue
• Site component
• Pleural effusion
Eosinophilic Granuloma

For Against
• Age • Site
• Lytic bone lesion • Small Soft tissue
• FDG Uptake component
Tuberculosis
For Against
• FDG avid rib lesion • Absence of lung lesions
• Lymphadenopathy • No necrotic nodes
• Splenomegaly
• Pleural effusion
Chondroblastoma
For Against
• Age • Site
• Cortical break
• No matrix calcification
Metastasis

For Against
• FDG uptake lesion • Young age
• Solitary lesion
• No known primary
• 1. Lymphoma
• 2. Plasmacytoma
Positive findings
• Difficulty in squatting and getting up from squatting position

• History of stiffness in upper limb and

• History of stiffness in lower limbs increased when trying to

squat or fold his legs, with difficulty in flexing knees
completely

• History of parasthesias and numbness on both plantar

surface of feet over toes and distal part of forefoot for 3
months
• History of a swelling over right infra scapular and
infra axillary region since 3 years, which was
gradually increasing in size slightly painful

• He complained of diffuse hyperpigmentation of face

and body for 1 year, more over soles and palms

• H/o loss of appetite and significant weight loss

(from 70 kg to 55 kg)
• History of SOB on exertion for 1 year, on climbing
stairs. Now since 3 months, unable to climb stairs
(both due to sob and weakness)

• History of joint pains involving both knee joints

and right ankle, increased on changing posture
from sitting to upright

• History of myalgias more over thighs than arms

EXAMINATION
• Pitting pedal edema typr Rt>lt
• B/l cervival lymphadenopathy and axillary
lymphadenopathy
• Swelling over rt chest wall with ill defined
margins, variable in consistency , soft firm
with mild tenderness
• Splenomegaly and hepatomegly – mild
• Wasting of both gluteal muscles

• Decreased bulk of left thigh

• Weakness of serratus anteror, rhomboids and

subscapularis (difficulty in overhead abduction)

• Proximal muscles weakness of lower limbs > Upper limbs

• Absent deep tendon reflexes

• Joint position sense impaired at bilateral 1st MTP

• Vibration sense lost at both malleoli

• MSK : TJ: rt shoulder , both knees and ankles

SJ: rt ankle

• ROM: rt hip flexion : terminal part restricted

• Lower limb spine mild tenderness

• Restriction of forward flexion due to stiffness

 Summary of investigations
• Low ESR (Normal ESR in males= age/2)

• Hypothyroidism

• Increased kappa and lambda light chain(normal ratio maintained)

• Minimal rt pleural effusion with pleural thickening

• Expansile osteolytic lesion of rt rib? Fibrous dysplasia

• Myositis
• ANA 4+ ?Connective tissue disorder

• PETCT s/o active expansile lytic lesion -?unlikely to be fibrous

dysplasia

• Demyelinating motor sensory neuropathy with muscle disease

ANALYSIS
 Generalised lymphadenopathy
• HIV infection • Castleman’s disease
• Mycobacterial infection • Kikuchi’s disease
• Infectious • Kimura disease
mononucleosis • Kawasaki disease
• Angioimmunoblastic T cell
• Systemic lupus
lymphoma
erythematosus
• Inflammatory pseudotumor
• Medications • Amyloidosis
• Sarcoidosis • Rosai-Dorfman disease 
• Lymphoma • IgG4-related disease 
 Hepatosplenomegaly causes
• Infective:
– Viral: Hepatotrophic (A,B,C,D,E) and other viruses (herpes,
cytomegalo, Ebstein-Barr, varicella,HIV, rubella, adeno,
entero, arbo.)
– Protozoal: malaria, kalazar, amoebic, toxoplasma
– Bacterial: sepsis; tuberculosis, brucellosis, syphilis,
– Helminths: hydatid, visceral larva migrans
– Fungal: histoplasmosis
 Hepatoslenomegay causes
• Metabolic:
Neiman-Pick, gangliosidosis, Gaucher, fucosidosis,
Wolman, glycogen storage, sialiodosis, galactosialiodosis,
amannosidosis.
• Malignancies:
– Leukemia, histiocytic syndromes, myeloproliferative
syndromes, lymphomas.
• Immunological:
– Chronic granulomatous disease, heriditory neutrophilia
• Developmental:
– Congenital hepatic fibrosis
• Congestive:
– Hepatic vein obstruction, constrictive pericarditis
 Arthralgias

• Inflammatory polyarthritis
• Viral arthritis
• Infectious
• Postinfectious or reactive arthritis
• Osteoarthritis
• Fibromyalgia
• Multiple sites of bursitis or tendinitis
• Soft tissue abnormalities
• Hypothyroidism
• Neuropathic pain
• Metabolic bone disease
• Depression
Pedal edema
Hyperpigmentation
 Lytic bone lesions

Plasma cell neoplasm- Multiple

myeloma Infections – tuberculosis,
brucellosis, histoplamosis
Skeletal metastasis

Lymphoma

Aggressive systemic mastocytosis Sarcoidosis

Langerhans cell histiocytosis Primary hyperparathyroidism

Causes of polyclonal gammopathies
Infections Viral : hepatitis, HIV, EBV, VZV

Bacterial : Endocarditis,
osteomyelitis, bacteremia,
tuberculosis
Connective tissue diseases Rheumatoid arthritis, Sarcoidosis,
Temporal arteritis
Hepatic Cirrhosis, Auto immune hepatitis,
PBC, PSC, Viral-induced hepatitis,
Ethanol abuse
Malignancies Solid tumours, ovarian tumours,
lung cancer, HCC, Renal tumours,
Gastric tumours
Causes of polyclonal gammopathies
Haematologic and Lymphoma, Leukemia,
lymphoproliferative disorders Thalassemia, Sickle cell anaemia

Other inflammatory conditions Bronchiectasis, cystic fibrosis,

chronic bronchitis, pneumonitis,
Graves, Hashimotos thyroiditis
Causes of monoclonal gammopathies

• Multiple myeloma
• MGUS
• SMM
• Plasma cell leukemia
• Solitary plasmacytoma
• Waldenstrom’s macroglobulinemia
• Heavy chain disease
• POEMS syndrome
• AL (light chain) amyloidosis
 ESR

High ESR Low ESR

• Infections • Extreme leucocytosis
• Connective tissue • Polycythemia
diseases • Hypofibrinogenemia
• Malignancy • Hypogammaglobulinemi
• Hypoalbuminemia a
• Renal insufficiency • Dysproteinemia
• Inflammation • RBC abnormalities
• Anaemia • Hyperviscosity
• Venous
thromboembolism
 Causes of demyelinating motor
sensory axonal neuropathy
• Diabetes • Monoclonal
• HIV gammopathy
• Critical illness • Autoimmune
• Uremic polyneuropathy • Toxic
• Amyloidosis  • HEREDITARY
• Hypothyroidism
• Vitamin deficiencies
• Lyme disease
Causes of proximal muscle weakness
• Myopathic and non myopathic causes
ANA 4+
Pleural effusion
 Differential diagnosis
Non malignant MALIGNANT
• Tuberculosis • Lymphoma
• Brucellosis • Leukemia
• Sarcoidosis • Metastatic carcinoma
• SLE • Multiple myeloma
• POEMS syndrome
• Waldenstroms
macroglobulinemia
 TUBERCULOSIS
FOR AGAINST
• Lymphadenopathy • Low ESR
• Lytic lesion of bone • No h/o TB contact
• Hepatosplenomegaly • Proximal muscle weakness
• Low back ache • Peripheral Neuropathy
• Increased gamma globulins( • ANA positive
normal FLC ratio) • Hyperpigmentation
• Arthralgia • No lung lesions
• Loss of weight
• Pleural effusion
Brucellosis
 Brucellosis
FOR AGAINST
• Lymphadenopathy • Low ESR
• Organomegaly • Proximal muscle weakness
• Neuropathy • ANA positive
• Lytic lesion of bone • Hyprpigmenttion
• Low back ache
• Increased gamma globulins(
normal FLC ratio)
• Arthralgia
• Weight loss
 Sarcoidosis
For Against
• Lymphadenopathy • Low ESR
• Arthralgia • Proximal muscle weakness
• Neuropathy • Hyperpigmentation
• Lytic bone lesion • ANA Positive
• Organomegaly • No e/o hilar
• Increased gamma lymphadenopathy
globulins(normal FLC ratio)
• Weight loss
 SLE
For Against
• Lymphadenopathy • Lytic bone lesion
• Arhtralgia • Low ESR
• Neuropathy • Anti dsDNA negative
• Increased gamma • No malar rash
globulins(normal FLC ratio
• ANA Positive
• Hyperigmentation (DLE)
• Proximal muscle weakness
• Organomegaly
 LYMPHOMA
FOR AGAINST
• LYMPHADENOPATHY • Hypothyroidism
• Organomegaly • ANA 4+
• Arthralgia • Proximal muscle weakness
• Neuropathy • Hyperpigmentation
• Increased gamma
globulins(normal FLC ratio)
• Lytic bone lesion
• Weight loss
 Leukemia
For AGAINST
• Lymphadenopathy • Absence of blasts in
• Organomegaly peripheral smear
• Neuropathy • Hypothyroidism
• Lytic bone lesion • ANA 4+
• Increased gamma • Proximal muscle weakness
globulins(normal FLC ratio) • Hyperpigmentation
• Arthralgia • Low ESR
 Metastatic carcinoma
FOR AGAINST
• Lytic bone lesion • Hypothroidism
• Lymphadenopathy • ANA Positive
• Organomegaly • Neuropathy
• Low back ache • Hyperpigmentation
• Arthralgia • LOW ESR
• Proximal muscle weakness
• Increased gamma
globulins(normal FLC ratio)
Multiple myeloma
 Multiple myeloma
FOR AGAINST
• Lytic bone lesion • Increased gamma
• Neuropathy globulins(normal FLC ratio)
• Low back ache • Hypothyroidism
• Organomegaly
• Proximal muscle weakness
• ANA positive
• Low ESR
• No A/G reversal
 POEMS syndrome
Mandatory criteria ( both required) Percentage
Peripheral neuropathy 100%
Monoclonal plasma cell disorder (almost always 100%
of the lambda light chain type)
Major criteria ( 1 required) Percentage
Osteosclerotic lesions 97%
Only sclerotic 47%
Mixed sclerotic and lytic 51%
Lytic 2%

Elevated VEGF levels 68%

Castleman disease (angiofollicular lymph node 15-25%
hyperplasia)
Minor criteria ( 1 required) Percentage
Endocrine abnormalities 67%
Skin changes 68%
Organomegaly 50%
Extravascular volume overload 29%
Thrombocytosis/polycythemia 50%
Papilledema 29%

Dispenzieri A, Kyle RA,et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101(7):2496-2506. doi:10.1182/blood-2002-07-2299
 POEMS Syndrome
FOR AGAINST
• Neuropathy • ANA positive
• Organomegaly • Increased gamma
• Hypothyroidism globulins(normal FLC ratio)
• Hyperpigmention • Proximal muscle weakness
• Lytic lesion( rare ) • Low ESR
• Low back acke
• Arthralgia
• Weight loss
• Pedal edema
• Pleural effusion
Waldenstroms macroglobulinemia
Waldenstroms macroglobulinemia
For Against
• Organomegaly • ANA positve
• Lymphadenopathy • Increased gamma
• Neuropathy globulins(normal FLC ratio)
• Lytic bone lesion • Proximal muscle weakness
• Arthralgia • Low ESR
• Weight loss • Hypothyroidism
• Hyperpigmentation
 Further Investigations
• Brucella antibodies
• Blood cultures
• SPEP
• IFE
• Urine for BJP
• Biopsy of lytic lesion of rt 9th rib
• BMA and Biopsy
• Excision Lymphnode biopsy
THANK YOU