Cotswold modification of Ann-Arbor Classification for Hodgkin lymphoma

For staging and prognosis of Hodgkin lymphoma.

Stage I	1 or 2 contiguous regions of lymph nodes on same side of diaphragm
Stage II	> 2 contiguous regions of lymph nodes on same side of diaphragm
Stage III	Both sides of diaphragm
Stage IV	Organ involvement*
X	Bulky disease >10cm
E	Extranodal involvement – used in conjunction with stage I-III
A/B	A: Absence of symptoms / B: Presence of constitutional symptoms

*Thymus and Spleen are lymphoid organ, thus included in the ‘lymph node’ involvement. They are not considered as ‘organ’ in this context.

Sarcoidosis

Sarcoidosis
Idiopathic. Granulomatous disease (non-caseating).
Systemic disease involving the lymph nodes, lungs, liver, heart etc.
Lung is the most commonly involved organ.

Clinical features:
- 3rd or 4th decades
- More in African american
- 70% have increased serum ACE - An indicator of the granuloma burden on the body

Nuclear Medicine:
- Gallium (Ga 67 scintigraphy) avid during active phase. No uptake when in remission.
Degree of uptake corresponds to degree of severity
Confirms diagnosis of sarcoidosis
Differentiate active alveolitis from inactive fibrosis
Signs:

1. Lambda sign: Hilar and Right paratracheal nodal uptake
2. Diffuse pulmonary uptake
3. Panda sign: Lacrimal and salivary glands inflammation

- PET scan: FDG avid during active phase. SUV can be >2.5

Acute Sarcoidosis

aka Lӧfgren syndrome
Clinical features:
- Fever, malaise, arthralgia +/- parotitis / uveitis
- Erythema nodosum
- Serum ACE level often increased
- Erythema nodosum
Has bilateral hilar lymphadenopathy, but absence of lung changes

Lungs:

Garland’s triad (1-2-3 sign)
Lambda sign on Gallium scan

1. Bilateral hilar lymphadenopathy
2. Right paratracheal lymphadenopathy

CXR:

-          Reticulonodular opacities, bilateral upper lobe dominant

-          Symmetric bilateral hilar, paratracheal, aortopulmonary window lymphadenopathy

HRCT:

-          Lymphadenopathy
-     1-5mm nodules in centrilobular and subpleural distribution
-     Upper lobe and peribronchovascular distribution
-     Irregular interlobular septal thickening
-     Architectural distortion

-          Advanced: Interstitial fibrosis- Peripheral honeycombing, Bullae
-     Some cases (rare): Alveolar sarcoidosis = Peripheral consolidation + Mediastinal and Bilat.hilar lymphadenopathy

Radiographic staging:
-     Stage 0: Normal CXR

-          Stage I: disease limited to mediastinal nodes

-          Stage II: involvement of lung and lymph nodes

-          Stage III: involvement of lung only

-          Stage IV: lung fibrosis

DDx:
- When advanced, can mimic progressive massive fibrosis (PMF)

Cardiac

Result in:
- Arrhythmia
- Ventricular aneurysms
- Sudden death

Head & Neck:

Involvement of glandular tissues, muscles, nerves.
1. Optic nerve and EOM hypertrophy and enhancement - uveitis
2. Parotid glands and lacrimal glands exhibits heterogeneous bilateral symmetrical enlargement - chronic dacroadenitis, parotitis

Gallium scan: "Panda sign" where there is increased uptake of the parotid and lacrimal glands (Looks like the whole panda sitting on the person's neck / replacing the head)

Neurological system:

Neurosarcoidosis, 5% of sarcoidosis patient
Usually lungs would have been involved already.
Granulomatous infiltration of the perineural tissue
Involvement of nerves, optic chiasm, hypothalamus, infundibulum, IAM
Presenting with cranial nerve deficits

Imaging:
Exhibit contrast smooth / nodular enhancements along the involved nerves, thickened leptomeninges, pachymeninges
Also enhancement in the perivascular space, ependyma
5% have solitary extra-axial mass

Complication: Hydrocephalus

Gastrointestinal:

Spleen:
Splenomegaly
Present as splenic granulomas, +/- calcification
If present = advanced stage of Sarcoidosis.
Spontaneous splenic rupture (rare)

Lymph nodes:
Mesenteric nodes

Musculoskeletal:

Affect small bones of hands and feet
Reticulated trabecular pattern (lace-like)
Cyst-like lesions
Destruction of terminal phalanges
Diffuse sclerosis of the vertebral bodies
Osteolysis in skull

Bonescan lesions

3 +/- 1 phase Bone scan
1st phase: Arterial blood flow; immediately after injection
2nd phase: Blood pool (tissue phase) 10min after injection
3rd phase: Delayed phase (Skeletal phase) 2-5hr after injection
4th phase: 24hr delayed phase if renal insufficiency and as a result suboptimal image on 3rd phase

Standard whole body bone scan: Acquire at delayed phase (skeletal phase)
Mechanism: Radiotracer adsorbed on hydroxyapatite matrix of the bone, where the mineral phase is active.

Superscan:
Increased radiotracer uptake of bone in relative to the soft tissue background and (almost) absent renal activity.
Causes:

1. Widespread metastasis in: Breast / Lung / Prostate CA, +/- Lymphoma +/- Bladder CA
2. Metabolic bone disease: Renal osteodystrophy, Osteomalacia, Hyperparathyroidism
3. Myelofibrosis, Systemic mastocytosis

Hot lesions:

1. Fibrous dysplasia
2. Paget's disease
3. Brown tumors
4. ABC (because it is very vascular)
5. Osteoid osteoma
6. Chondroblastoma
7. Metastasis (Osteoblastic activities ie Sclerotic eg Prostate CA mets)
8. Joint diseases
9. Dental infection
10. Acute fracture (after 24 - 48hr)

3 phase positive lesions: (lesions that causes bone remodelling)

1. Osteomyelitis
2. Healing fracture
3. Orthopaedic implant
4. Neuropathic osteoarthropathy

Normal scan: (no increased or decreased uptake)

1. Acute fracture (acute phase)
2. Bone cyst, bone island, exostoses
3. Multiple myeloma ** there for this condition is monitored by skeletal survey radiograph**
4. Osteoporosis

Cold lesions:

1. Radiotherapy
2. Avascular lesion
3. Multiple myeloma 
4. Haemangioma
5. Advanced carcinoma

Varying lesions

Mixed lytic/sclerotic metastasis (Breast, Lung etc) may show hot or cold (with hot margins).

Enostosis - Bone island

Commonly known as bone island

Mature cortical bone within the medulla
Due to failure of resorption during endochondral ossification
Asymptomatic

Features:
CT/Radiograph: 
- Spiculated margin - blends with the surrounding trabecular bone
- Dense sclerotic focus
MR:
- All sequences: Very low SI
Scintigraphy
- Typically no activity. Some may have increased activity

DDx:
- Dense osteoid osteoma
- Osteoblastoma

PVNS

Pigmented villonodular synovitis
- Unknown aetiology
- Synovial proliferation disorder
- intracellular Haemosiderin deposition (thus "Pigmented")

Monoarticular
Age 30-40s

Benign proliferation of synovium in the joint or in bursa/tendon sheath +/- hemorrhagic effusion
If occurs in the tendon sheath => then it is known as Giant Cell Tumor of the tendon sheath

Clinical features:
- Insidious onset
- Monoarticular arthropathy
- Swelling
- Stiffness
- Pain

Pathology:
- Hyperplastic synovium
- Lipid-laden foam cells, histiocytes, giant cells, haemosiderin deposits

Distribution:
- Knee > Hip > Ankle > Shoulder

Radiograph:
- Dense effusion
- Dense soft tissue mass (due to haemosiderin)
- Bone erosion
- Large subchondral cysts
- Preserved joint space
- Typically normal in the early stage

MRI:
- Joint effusion
- Focal / diffuse synovial thickening: Low signal T1w and T2w, and blooming artifact on GRE (due to hemosiderin deposition)

DDx:
- (repeated) Haemarthrosis such as Haemophilics
- Septic arthritis
- Articular amyloid deposition (similar in MRI appearance)

Rx:
- Synovectomy (~50% with local recurrence)

Bosniak classification for Renal Cyst

Applicable to US, CT or MR imaging.

	Features	Management
Bosniak I	Anechoic cyst Thin walled No septa, calcification, solid components No contrast enhancement	No intervention required	Class 1 Renal cyst
Bosniak II	Hairline-thin septa Fine calcification / Short thick calcification No contrast enhancement	No intervention required	Class 2 Renal Cyst Class 2. Notice the faint thin septa? Also a smaller simple cyst in the lower pole.
Bosniak IIF	Multiple hairline-thin septae +/- minimal thickening Minimal wall thickening Thick / Nodular calcification No soft tissue component	Needs follow-up
Bosniak III	Thick wall / septa, irregular / smooth	Needs surgical intervention	Class 3. Notice the thicker septa and larger size of the cyst? Class 3 Renal Cyst
Bosniak IV	Bosniak III + soft tissue component which enhances.	Clearly malignant

Psoriatic arthritis

Psoriatic arthritis

Polyarticular arthritis
Skin disease: Psoriasis (Skin rash)
Chronic inflammatory
40yo, Caucasian
?Factors involved: Genetic, Immune system, Environmental
HLA-B27 +ve in 60%

Hand:
- Most commonly involved skeleton
- Interphalangeal joint (PIJ, DIJ)
- 'Fluffy periostitis'
- Bony proliferation*
- Joint erosion from articular margin* to centrally.
- Tapering of one end, saucerisation of the other end of the articular surface: Pencil-in-cup deformity
- Preserved bony mineralisation (no osteopenia), ivory phalanx
- Ankylosis
- Diffuse soft tissue swelling: Sausage digit

SIJs:
- Asymmetric sacroiliitis, sclerosis, large erosion
- Ankylosis is UNcommon

Vertebra:
- More commonly involved in MALE
- Asymmetric
- Atlanto-axial subluxation
- Squarring of vertebrae
- Enthesitis: comma-shaped ossification, paravertebral (parasyndesmophytes)
- Thoracolumbar region

Others:
- MTPJ
- TA insertion of the Calcaneum: Fluffy periostitis & Erosion

*HALLMARKS for Psoriatic arthritis

DDx:
- Ankylosing spondylitis (Ossification less bulky, symmetric sacroiliitis)
- Reactive arthritis (Genitourinary symptoms)
- Hyperparathyroidism (similarity: presence of large SIJ erosion)