Transcript CASE OF THE MONTH - Institute of Medicine, Nepal
CASE OF THE MONTH
DR. SHAILESH MANANDHAR PAEDIATRIC RESIDENT 1
ST
YEAR IOM
Patient’s Profile
Name : Kapindra Regmi Age / Sex – 12yrs / M Add : Dolkha I.P No : 12153 DOA : 2062/7/12 at 9:30 pm DOD : 2062/8/8 at 11:00 am Admitting Diagnosis: Lt. Pyopneumothorax with neck mass ? Lymphoma Final Diagnosis : Malignant thymoma Stage 3 with Lt. chylopneumothorax
Chief complains
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Progressive difficulty in breathing for 10 days Difficulty in swallowing for 10 days Upper abdominal swelling for 5 days
History of Present Illness
Patient was apparently well 20 days back. Then , he developed mild to moderate grade fever intermittent not a/w chills & rigor but with profuse night sweats lasting for 10 days.
Cough with mucoid expectoration, no hemoptysis, no vomiting, no dysuria, no pain abdomen Chest pain more on left side and on coughing.
Complained throat pain during swallowing Taken treatment for local medical shop tab. Ofloxacin 200mg BD for 7 days – fever subsided but cough not improved
HOPI Contd…………..
Subsequently mother noticed swelling in neck ; followed by swelling of upper abdomen couple of days later. Gradual difficulty in breathing and swallowing Bowel and bladder habit were normal No noisy breathing No H/O joint pain or swelling, rashes, swelling of legs, no hoarseness of voice, no jaundice.
No h/o bony tenderness.
No H/O loss of consciousness or seizure
HISTORY OF PAST ILLNESS
Never needed medical care and medication.
FAMILY HISTORY
Only child in family Mother: 35years/ House-wife Father : expired 4 years back in RTA Low socio economic class family Mother earn living by working in own small field and other people’s houses in village No H/ O similar illness/ TB in the family or in close contacts.
No H/O consanguinity of marriage.
No H/O pet in the family.
BIRTH HISTORY -
FT/S/F/NVD at Home . Ante-natal/intra-partum or postpartum period: Uneventful
IMMUNIZATION HISTORY –
not immunized
DEVELOPMEENTAL HISTORY -
Normal for his age
NUTRITIONAL HISTORY
Taking less than required calorie.(80 Kcal/kg/day )
TREATMENT HISTORY
Admitted In Kathmandu Model hospital, Dolkha one week back. Inj. Ceftriazone 50mg/kg/d q 12hr for 5 days History of aspiration of thick white fluid from left chest twice.
Referred from there with discharging diagnosis of left pyopneumothorax with neck mass ? Lymphoma to cardio thoracic surgery department for evaluation and management of neck mass and chest tube insertion.
ON EXAMINATION
Child was conscious oriented to time, place and person but looked dyspneic and tachypnic.
Vitals: Pulse: 116b/min normo volumic R/R : 48/min regular B.P : 90/40 mm Hg Temp: 100 0 C Anthropometry: wt. 25 kg Ht. 136 cm wt. for age : 62.5 % of expected ht. for age : 90 % of expected wt. for ht. : 83.3 % of expected
Puffy face with mild submandibular region swelling.
Distended neck veins.
Visible swelling on anterior lower part of neck. On palpation hard fixed mass approx. 3 cm by breadth ill defined borders arising from retrosternal region. Can’t get lower border of mass.
Neck mass –doesn’t move on deglutition/ protrusion of tongue.
Lymphnodes- Single Rt. Axillary 1*1 cm, multiple cervical and submandibular 0.5 to 1cm Pallor / jaundice / cyanosis – absent Clubbing- absent Pedal edema – absent.
Ear/nose and throat examination- normal Joints : Normal Bony tenderness: Absent Non pitting edematous swelling of anterior chest and upper abdomen.
RESPIRATORY EXAMINATION
INSPECTION- Fullness in Lt. anterior chest wall. Reduced movement on same side. PALPATION- trachea- deviated to left side. Reduced tactile and vocal fremitus in Lt. side on lower half.
PERCUSSION- stony dullness on Lt. lower part of chest.
Hyperresonant note on Lt. upper part of chest.
AUSCULTATION- absent BS on Lt. side of chest. Normal vesicular breath sound with no added sound on right side of chest.
PER ABDOMEN :-
Inspection: mild distension of upper part of abdomen; visible veins with flow up to downward at supraumbilical region, central umbilicus. No scar marks, No visible peristalsis Palpation: No tenderness on superficial or deep palpation. Liver/Spleen non palpable. No abdominal LN palpable.
Hernial orifices: Intact Both testes were palpable at lower end of inguinal canal normal size with intact sensation.
Percussion: Tympanic. No fluid thrill or shifting dullness Auscultation: normal bowel sound
CVS : apex beat localized at 4 th ICS 3cm medial to left nipple. S1,S2 – N , No murmur CNS : - conscious, cooperative and oriented to time, place and person. - cranial nerves grossly intact - muscle tone/power/bulk were normal in all limbs - no involuntary movements - superficial and deep tendon reflexes N - Sensory examination with in normal limit - Normal Gait.
PROVISION DX
:
Lt. Pyopneumothorax with neck mass ? Lymphoma ? Tuberculosis INVESTIGATIONS
CBC:
Hb: 13.4 gm% TC: 12,100 (N-75, L- 15, E-8, M- 2) Platelet:160,000/mm ESR : 12 Na: 139 K: 4.5m mol/L Urine R/E: normal
Chest tube was inserted on left side - whitish turbid fluid ~ 2.5 litre drained and admitted in Medical Ward.
Inj. Cloxacillin 100mg/kg/d Inj. Amikacin 15mg/kg/d Tab. Paracetamol 6hrly
D3 of admission
Reduced dyspnea, puffiness of face and look comfortable.
Tem. 100 0 F PR 102/m B.P: 96/60 mm Hg Chest tube drain ~ 1.5 lit of milky white fluid with little dirty white fibrinous sediments .
Eating well .urine - N INV: Pleural Fluid Analysis TC: 21,900/cumm (L- 91%, N- 9%) protein – 61 micromol / L
A> Lt. chylopneumothorax ? Lymphoma ? Tuberculosis Plan- pleural fluid triglyceride level sr.creatinin, urea, LFT Sputum for AFB Mantoux test
D4,D5 of admission
Complains cough and chest pain otherwise comfortable.
Vital stable still have low grade fever.
Non pitting edema of left upper limb noted.
Chest tube draining more milky fluid upto 2.5litres.
No dehydration, passing urine.
Air entry on left side present.
INV - pleural fluid triglyceride level – 9.3 m mol/L ( 0.5 – 1.8 m mol/L) Sr.creatinin – 0.4mg/dl Urea – 20 mg/dl.
Sr. albumin 2g% Total protein – 3g% SGPT 15IU SGOT 16 IU
Blood C/S – sterile after 96 hrs.
Pleural fluid C/S- E.coli isolated sensitive to Imipenam Intermediate sensitive to Amikacin resistant to Ampi, cipro, genta, ceftazedime, tobramycin, cephalexin, cotrim.
USG - Multiple enlarged LNs in neck, mediastenum and clavicular region with left pleural effusion.
Liver- enlarged in size, no SOL other viscera WNL.
X- ray chest Lateral view Plan for neck mass / lymph node biopsy.
D6
Patient’s condition static Mx – no induration Sputum for AFB- Negative.
Advice to take high protein diet.
D8 – D10
Looked better Fever subsided, still complains cough.
Neck swelling and venous prominence reduced than admission day.
Neck mass felt softening than before.
Chest tube functioning; draining still 1 -2 lit/day
D12
Case referred to CTVS department, TUTH for expert opinion and further management. Case seen by Dr. P. Sayami, with impression of mediastinal lymphoma with left chylothorax, USG guided FNAC of mediastinal mass and follow up with report was adviced.
D15
USG guided FNAC of neck mass done – Mixed population of mature and immature lymphoid cells; with few clusters of atypical epithelial cells – moderately Pleomorphic, oval to spindle nuclei and scanty cytoplasm and few squamous cells in background. Impression: Malignant Thymoma.
Plan for CT scan thorax Patient party counseled about the disease and possible therapy and poor prognosis.
D17 – D20
Patient look dysneic with puffy face.
Complaining of dysphagia more for solid food Temp. 101 0 F PR 120/m BP- 90/50 mmHg No Ptosis, no diplopia, no muscle weakness No rashes, joint pain.
Chest - absent BS on left lower 2/3 rd .
Chest tube not draining since yesterday.
Chest tube irrigation done and Chyle started draining.
D21
CT scan thorax-
Large lobulated soft tissue density well defined mass in anterior superior mediastenum that shows heterogenous enhancement with contrast; encasing all the major vessels of the mediastenum including aortic arch and pulmonary arteries . Thick irregular enhancement of pleura with nodular thickening at places.
Impression- Malignant thymic mass
D22
Consultation with CTVS team was done for further management of case with CT report.
With impression of advanced thymic carcinoma – inoperable, case was referred for Radiotherapy from their side.
D23
Patient general condition- same Patient party were given option for treatment after explaining prognosis of disease.
Referred to Bhaktapur Cancer Hospital for further treatment.
D25
Patient came back from Bhaktapur Cancer Hospital with a referral letter to oncology department. Patient was advised palliative chemotherapy of Ipbosphamide and adriamycin and asked to come on follow up for radiotherapy.
D26
On oncology department patient party again counseled about the poor prognosis of disease and possible expenses.
Then, they refused treatment.
Discharged on request on 2062-08-07 WITH FINAL DIAGNOIS – Thymic carcinoma ( stage 3) with Lt. chylopneumothorax & superior vena cava syndrome without obvious paraneoplastic syndromes.
THYMOMA
ANATOMY :
Mediastinum – pleural cavity laterally Thoracic inlet superiorly Diaphragm inferiorly .
Anterior
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Thymus, Fat and LN Middle – Heart, pericardium, ascending and transverse aorta, branchiocephalic veins, trachea, bronchi, LN Posterior – descending thoracic aorta, esophagus, azygos vein, autonomic ganglia and nerves, thoracic LN
Differential diagnosis of mediastinal mass by anatomic location
ANTERIOR
Thymoma
Teratoma/seminoma Lymphoma Parathyroid adenoma Intrathoracic goiter Lipoma Lymphagioma Aortic aneurysm MIDDLE Lymphoma Pericardial cyst Broncogenic cyst Metastatic cyst Systemic granuloma POSTERIOR Neurogenic tumor Bronchogenic cyst Enteric cyst Xanthogranuloma Diaphragmatic hernia Meningocele Paravertebral abscess
THYMUS :
Soft, bilobed, pyramidal organ in anterior superior mediastenum At birth wt. 10 -15 gm Anteriorly – sternum, sternohyoid, sternothyroid muscles Posteriorly – pericardium, arch of aorta & it’s branches, left branchiocephalic vein, trachea.
One of two primary lymphoid organ of body.
Provides unique microenvironment in which T cell precursors undergoes development, differentiation & clonal expansion During this process, exquisite specificity of T cell responses is acquired; also their immune tolerance to body’s own components.
Embryologically, thymic epithelium is derived from both the ectoderm and the endoderm of the 3 rd & 4 th branchial cleft and pharyngeal pouches.
Histology of Thymus:
INTRODUCTION
Thymoma is a tumor arising from epithelium of thymus gland.
Majority of thymoma looks histological benign.
Usually follows indolent course patient surviving for years.
34% of thymomas invades their own capsules, extending to surrounding structures.
Less than 10% behave like invasive epithelial malignancies
Epidemiology
Incidence 0.15 cases per 100,000 accounting 0.2 to 1.5% of all malignancies
( reference Tumor of mesiastinum by ACP. Dec 15 05’)
Thymoma constitute about 50% of anterior mediastenal neoplasm in adult, Lymphoma -25% Peak age of incidence – 4 th to 6 th decades of life Sex – no predilection of gender Children very rarely affected.
Usually associated with the host of unusual paraneoplastic syndromes
PATHOLOGY:
Tumor arises from epithelial component of thymus.
Most of them are solid tumor; but up to one third may have components that are necrotic, hemorrhagic or cystic.
Histologically, 3 groups of thymic tumors can be distinguished : 1.
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Typical thymoma - with no cytological features of malignancy Atypical thymoma - with organotypic features of thymoma but with areas of atypia and occasional mitosis (WDTC).
Thymic carcinoma- with abundant mitotic figures and other cytological features of malignancy.
Masaoka
Staging of Thymoma (1981)
Stage
1 2a 2b 3
Degree of invasion
Macroscopically completely encapsulated with no microscopic extracapsular invasion Microscopic invasion through the capsule Macroscopic invasion into mediastenal fat or pleura.
Invasion into adjacent structures (pericardium, great vessel or lung)
5yrs survival rate %
96 – 100 % 86 – 95 % 56 -69 % 4a 4b Pleural or pericardial metastasis Lymphatic or hematogenous metastasis 11 – 50 %
WHO Classification of Thymoma
(1999)
CLASS CYTOLOGIC FEATURES
Type A Type B Type B1 Type B2 Type B3 Type C Spindle shaped, Medullary Mixed Lymphocyte rich, lymphocytic, predominantly cortical, organoid.
Cortical Epithelial, atypical, squamous, well differentiated thymic carcinoma Thymic carcinoma
( Referance - cancer treatment Review 2000 ; 26: 127- 131)
CLINICAL MANIFESTATIONS
:
One third to one half of patient present with asymptomatic mediastinal mass on chest radiography.
one third present with local symptoms related to intrathoracic mass.
One third are detected during evaluation of Myasthenia gravis.
Distant metastasis are rare at initial presentation.
When present, most common metastatic site is Pleura
Common symptoms at presentations are:-
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Cough - 60% Chest pain - 30% Fever / Chill - 20% Dyspnea - 16%
( reference Tumor of mesiastinum by ACP. Dec 15 05’)
At presentation ~ 40 % of thymic tumor – Stage 1 ~ 25 % of thymic tumor – Stage 2 or 3 ~ 10 % of thmic tumor – Stage 4a < 1 % of thymic tumor – Stage 4b
( Reference -Thymic tumors – review article Ann Thorac Surg 2004 ; 77 : 1860 – 9 )
Localizing symptoms secondary to tumor invasion of surrounding structures
Involved Anatomic structures
Bronchi / Trachea Esophagus Spinal cord/vertebral column Recurrent laryngeal nerve Phrenic nerves Stellate ganglion Superior vana cava
localizing symptoms
Dyspnea, post obstructive pneumona, atelactasis, hemoptysis Dysphagia Paralysis Hoarsness, vocal cord paralysis Diaphragmatic Paralysis Horner’s syndrome Superior Vana Cava Syndrome
Paraneoplastic syndromes associated with Thymoma
(Thymoma- state of art, jour of clin oncol 17 : 2280 – 2289 by American society of clinical oncology) Myasthenia gravis – most common (30 – 50%) Pure red cell aplasia Acquired hypogammaglobulinemia Auto immune hemolytic anemia Agranulocytosis Peripheral neuropathy Pernicious anemia Limbic encephalopathy Nephrotic syndrome Alopecia areata
Contd…
Panhypopituitarism Hyperthyroidism Addison’s disease Rheumatoid arthritis SLE Systemic sclerosis Sarcoidosis Dermatomyositis Inflammatory bowel disease & Whipple’s disease Hypertrophic osteoarthropathy
CxR CT scan MRI
DIAGNOSIS
FNAC of tumor – USG/ CT guided.
Excisional Biopsy
TREATMENT
1.
Surgery- mainstay of treatment because majority of these tumors 90 – 95% are localized.
Stage 1 – completely resectable Stage 2 – 42 – 100 % resectable stage 3 – 0 to 89 % resectable Stage 4 - 0 to 78% resectable (
Jour of onco vol 17,no 7(july)1999
) Extensive resections seem justifiable since complete resection is probably most important prognostic factors recurrence rate is less than 2% and 28% resp. for non – invasive and invasive tumors (
cancer treatment review 2000;26:127 -131)
Adjuvant Radio therapy – moderatively radio sensitive.
a retrospective study has shown that without post – operative radiation for invasive thymoma, the relapse rate was 26%.
10 years survival rate after non – invasive thymomas range between 67 – 80 % while those for invasive disease range from 35 – 53 %
Chemotherapy- candidates for cytotoxic chemotherapy are - who presents with metastatic disease not amenable to local treatment modalities.
- surgery and/ or radiation therapy fails.
Combined modality therapy- advanced invasive thymoma.
Salvage therapy- somatostatine analogues used in chemotherapy refractory/ recurrence cases.
Indium labelled octreotide and prednisone.
High dose chemotherapy and stem cell support – investigational approach.
Novel therapies
Recent chemotherapeutic agents – - taxoids, topoisomerase 1 inhibitors and gemcitabine - somatostatine analogs - High dose chemotherapy with stem cell rescue - Investigational agents – flavonoids, signal transduction inhibitors, and anti – angiogenic agents
Survival rate %
Stage 1 2 3 4 5yr 71- 100% 60 – 98% 46 – 89% 40 – 71% 10yr 87- 100% 42 – 98% 26 – 78 % 0 - 47% 15yr 78% 73% 30% 8% ( Kunda and MOnden, Regnand et al, Maggi et al, Verley and Hollman, Comen et L, Nikin et all, Monden et al, Blumber et al, Rruffinin et al, Quintanilla – Martinea et al)
References
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Tumor of mediastinum, by American college of physicians; December 15 2005;128 www.chestjournal.org
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Malignant thymoma: current status and future directions – P.N. Lara Cancer treatment Review 2000; 26; 127 -131 Thymoma; state of Art ( review article ) by Charles R. Thomas, Jr. , Cameron D. , Patrick J. Lochrer, Sr.
Thymic tumors ( review article ) by Franck C. Detterback, MD Ann Thorac Surg 2004; 77 : 1860 – 9 Classification of Thymic neoplasm ( original article ) – Desai Saral et al Indian Journal of surgery 2004 vol. 66, issue 2; 93 – 96.
Thymoma – pathological study of 50 cases ( original article ) Journal of postgraduate medicine, 2004 vol. 50, issue 2, 94 -97.