-Prof.U.

Bhattacharya

INTRODUCTION

Clinical expression of parathyroid neoplasms are not due to physical enlargement of the glands but because of excess hormonal effects.

TYPES OF HYPERPARATHYRIODISM

Primary hyperparathyroidism:
*parathyroid glands primarily affected- seat of neoplasms/hyperplasia.Autonomous state of hormone hyperproduction. *secondary effect- hypercalcaemia.

Secondary hyperparathyroidism:
*parathyroids secondarily stimulated by chronic persisting hypocalcaemia.

Tertiary hyperparathyroidism:
*chronic secondary stimulation of parathyroids leading to an autonomous, uninhibited state of hormone production.

PATHOLOGICAL ENTITIES OF PRIMARY HPT

TYPE OXFORD SERIES % WORLDWIDE SERIES %

Single adenoma Multiple adenoma Multiple gland hyperplasia Carcinoma

84 02 14 1.5

70-93 2-24 3-26 1-4

TARGET ORGAN EFFECTS OF PTH

Bones:
-Resorption *osteoclastic resorption. *osteocytic osteolysis. -Deposition *osteoblastic stimutation. -increased calcium absorption through distal tubules. -synthesis of activated form of vitamin D. -stimulation of Adenyl cyclase system of tubular epithelial cells leading to increased c-AMP in urine. -cyclic AMP mediated phosphaturia or bicarbonate [Effect: hypophosphataemia, hyperchloremic acidosis] -Hypercalciuria- due to increased calcium load in urine.

Kindey:

INCIDENCE OF PRIMARY HPT

New cases per 10,000

Age

Men 05

Women 08

<40

40-50
>60

26
92

104
189

SINGLE GLAND-MULTIPLE GLAND DISEASE

Pathological aspect:
*unreliable pathological description.

*vague distinguishing features.

*Parathyroid tissue is identified with certainty. *per-operative visual inspectionmost reliable.

CLINICAL MANIFESTATIONS
Bones
Bone pain Pathological fracture Joint pain Osteitis fibrosa cystica- associated deformities.

Kidney

Stones Renal colic Uraemia Polyuria.

G.I tract

Weight loss Anorexia Nausea, vomiting Constipation. Polydipsia Abdominal pain Peptic ulcer Pancreatitis Gall stones.

Clinical manifestations
Neuro-psychiatric symptoms
Lethergy Muscle weakness Headache Confusion Hyporeflexia Ataxia Depression Psychosis Hearing loss.

Cardiovascular system

Hypertension Heart blck Bradycardia.

Others

Pruritus Thrombosis Myalgia.

Radiological features

Bones:
*generalised bone density loss *subperiosteal bone resorption *tufting of terminal phalanges *bone cysts *brown tumours *rugger-jersy spine *pepper-pot skull.

Kidney:

*renal stones *nephrocalcinosis.

Metastatic calcification of

skin, blood vessels, joints.

Radiological features

Diagnosis

Hypercalcaemia is to be established. Correction of serum calcium level as per existing serum albumin level. Formula: Corrected serum calcium= [measured serum calcium + (40A)] *0.02 (mmol/L) A= serum albumin (g/L) Demonstration of increased PTH. Hypophosphataemia or hyperchloremic acidosis Serum chloride/ serum phosphate > 30 is highly suggestive. Increased 24 hrs. urine calcium excretion.

Increased c-AMP in urine.

Common causes of hypercalcaemia

Malignancy Vitamin D intoxication Thiazide diuretics Hyperthyroidism Milk- alkali syndrome Sarcoidosis Familial hypocalciuric hypercalcaemia Immobilisation Lithium therapy Addisonian crisis.

Pre-operative localisation of parathyroids

High resolution real-time ultrasonography. CT localisation. Thallium-techetium sabtraction scan. Sestamibi radionuclide scan. Selective venous sampling with PTH assay. Selective angiography.

Localisation techniques, particularly the invasive ones are followed for re-exploration procedures- not for initial procedures.

Surgical plan
Indications

All symptomatic patients Asymptomatic paients

markedly elevated serum calcium. life threatening hypercalcaemic episode. reduced creatinine clearence. detection of renal stone. markedly elevated 24 hrs. urine calcium. substantially reduced bone mass or when - pt. is young (<50yrs.) - pt. is not follow-up able - co-morbid condition may aggravate later.

Surgical plan
principle

Confirmed diagnosis Patience Patient counselling All 4 glands are to be visually inspected or judged for enlargement/ disease.

Surgical procedure
glandular search

At normal locations.

Missing glands
- thyrothymic tissue

- thymic rests in mediastinum - alongside or deep to oesophagus - within thyroid lobe

Intraoperative USG may be helpful.

Surgical procedure
extent of surgery

Single gland disease (adenoma)

- excision of the diseased gland

Multiple 2/3 gland disease (adenomas)

All 4 glands disease

- excision of the diseased glands

- subtotal parathyroidectomy

or,

total parathyroidectomy with autotransplantation of parathyroid tissue into nondominant forearm (particularly for MEN I disease)

Surgical procedure
auto implantation of parathyroid tissue

Parathyroid tissue mined to 15-20 pieces. Confirmed by frozen biopsy/ cryopreserve (few pieces) Implanted into non dominant forearm muscle and marked. Antecubital vein blood sampling for PTH. If necessary, cryopreserved tissue implanted.

Persistant or recurrent hyperparathyroidism

Missed diseased gland Regrowth of diseased tissue Auto implantation of broken tumour tissue Parathyroid carcinoma.

Adjunctive procedures:
- pre operative / peroperative localisation
* selective venous sampling with PTH assay * intra operative ultrasound.

- urinary c-AMP estimation to document successful resection.

Parathyroid carcinoma

Rare. Dense periglandular adhesions. Invasion of surrounding structures. Spread to lymph nodes and distant metastasis pathognominic. Difficult histologic interpretation- capsular and angio invasion. Parathyroidectomy, ipsilateral thyroid lobectomy & modified neck dissection. Succumd to uncontrolled hypercalcaemia.

Acute hypercalcaemic crisis

Serum calcium more than 3 nmol/L. Life threatening- cardiac arrest, acute renal failure. Vomiting, dehydration, confusion and coma. Rehydration or frusemide diuresis. (not thiazide) Dialysis. Drugs:
- calcitonin - glucocorticoids - mithramycin - biphosphates.