Mrunal M Bhavsar f

f

Case of Pleuropulmonary Blastoma
Embryonal Rhabdomyosarcoma

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My journey towards heavenly "Akshardham".
1-Jan-2000 to 16-Feb-2000 (47 Days)

1-Jan-2000 to 6-Jan-2000

First symptoms had been seen were cough, respiratory difficulty and remittent fever for these days. I was gradually becoming weak.

7-Jan-2000

Even after prescribing antibiotics by our family physician Dr. Chandulal M. Bhavsar, no improvement was found in my health. Looking so he suggested having chest X-ray.

X-Ray Report : "There is massive pleural effusion on the right side with displacement trachea and heart to left. There is slight prominence of vascular markings in the left hilar region. Left costophrenic angle appears clear. Further follow up examination would be of help to note the progress."

Dr. Narendra L. Patel
D.M.R.E., D.M.R.D. (ENG) F.I.C.R.

8-Jan-2000

I was admitted at the hospital of Dr. Ramesh S. Vora (M.D. D. Ped.). He tapped the fluid and sent for examination.

Culture Report :
Grams Reaction : No organisms were seen.
Ziehl Neelsen Stain : 1 to 2 acid fast bacilli per 100 field seen
Negative Stain : No fungal elements were seen.
Organisms Isolated : No growth observed even after 48 hours of incubation.

Dr. Dilip N. Zaveri
M.Sc. (Medical) Gold Medalist

10-Jan-2000

Having pulled out approx. 800 cc. pleural fluid, Dr. Vora found my right lung as it was on 8th. He immediately told to take abdomen sonogram.

Sonogram Report : Massive right pleural effusion noted. There are multiple hypoechoic masses of 32 mm, 4 mm & 92 mm size attached to pleura & right dome of diaphragm, suggesting pleural masses, biopsy exam. would be helpful.

Dr. Vikram Patel
M.D., D.M.R.E.

In the evening, I was moved to Dr. Tushar J. Shah (M.S.; M.Ch.) to insert ICD with frighten. Dr. Tushar Shah had also pulled out approx. 450 cc. Pleural fluid and forwarded it for Cytology and Histology reports.

Histology Report :
Micro Description : Smears and cell block reveal red blood cells, neutrophiles and lymphocytes.
Diagnosis : Hemorrhagic material.

Dr. Bharti. N. Parikh
M.D. (Path.) U.S.A.

Cytology Report :
Microscopic Examination : Smears show mainly RBCs with few chromic inflammatory cells and mesothelial cells. No atypical cell identified.
Conclusion : Negative for malignant cell.

Dr.Nalini J. Shah
M.D. (Path & Bact.)

To night, I noticed everybody around me worried. I couldn’t even sleep for more than five minutes whole night due to ICD.

11-Jan-2000

In the morning Dr. Shah observed the flow of fluid came out entire night, it was again approx. 400 cc., and suggested to have CT Scan of Thorax.

CT Scan Report : The CT findings show large soft-tissue density mass lesion in right hemi-thorax involving pleural cavity and extending into adjacent posterior mediastinum, compressing cardiac chambers with compressive collapse consolidation of right lung.
Possibility of malignant pleural mass should be considered.
? Lymphoma. ? Soft-tissue sarcoma.

Dr. Kalpesh Shah
M.D., D.M.R.E.

This was the unfavorable report and inauspicious moment for my family. Dr. Shah doubted of cancer and advised to immediately rush to concern doctor.

12-Jan-2000

I again shifted to The Gujarat Cancer & Research Institute (M. P. Shah Cancer Hospital) in the morning under treatment of Dr. P. M. Shah. I was given 400 cc. Blood support. X-Ray, Sonogram, LFT, Bone marrow and several other investigations had been performed. Some among those were :

Bone marrow Report : Uninvolved.
Special Investigation : Serum Uric Acid Estimation : 11.80 mg/dl
Serum Calcium Estimation : 8.00 mg/dl
Sr. L.D.H. : 3160.6 (I. U. / Litre)

13-Jan-2000

Review of CT abdomen and CT guided biopsy was performed under high risk consent.

CT Scan Review : There is a mixed density complex necrotic tumor seen involving the rt. hemi thorax more of middle and lower zone with homogenous enhancement and tumor necrosis seen within the mass lesion. There is an erosion of lower dorsal vertebrae on rt. side with intraspinal extension of tumor seen. No evidence of mediastinal nodes seen. Lt. lung appears normal. No evidence of pleural effusion is seen.
Comments :  Appearances are more in favour of Neuroblastoma.

Dr. Shreedevi B. Patel

Cytology Report :
Material sent : FNAC
Site of Aspiration : CT guided bx. from mediastinal mass
Malignant : Small round cell tumor. Further typing is not possible. Sug. Bx. & HPE are recommended for definite typing.

Dr. Ilesh Safi

Surgical Pathology Report :
Gross Examination : Specimen consist of multiple, brownish, soft tissue pieces.
Diagnosis : Section shows blood clot, necrosis & few mature lymphocyte. 
No evidence of malignancy.

Dr. Priti Trivedi

15-Jan-2000

Abdominal enlargement seen due to Ascites. Atrophy of lower extremities with oedema on feet.

Serum Ferritin Report : 210 ng/ml

Dr. Ashwin C. Patel
M.S. Biochem (U.S.A.)

21-Jan-2000

At a standstill, perplexity was going on. No improvement had been seen. I was becoming more & more weak. But, I could eat everything till date.

Report : Sr. L.D.H. : 580.7 (I. U./Litre)
VMA Report : 1.5 mg. / 24 hour

22-Jan-2000

As per advice of Dr. P. M. Shah & looking to my condition chemotherapy of Neuroblastoma (OPEC Protocol) had been started.

24-Jan-2000

Tumor Markers : 1. Human Chorionic Gonadotrophin (HCG B) : < 0.01 IU/ml.
2. Alpha Fetoprotein (AFP) : 3.85 ng/ml

25-Jan-2000 to 27-Jan-2000

During these days, no improvement was seen. All the family members were embarrassed. All were ready to move heaven & earth for my good health, but nothing was there in their hand. All were praying to God. Some times they chocked with emotions also.

28-Jan-2000

As, no definite diagnosis came out, doctors decided to take USG guided bx. Again, they wanted not to, but no way, I was given anesthesia under high risk.

Surgical Pathology Report :
Gross Examination : Specimen consist of multiple, gray-white, soft to firm tissue pieces.
Diagnosis : Necrotic tissue & atypical round to large polygonal cells, myxoid change fibrous stroma are seen. From imaging study & histological appearance, as well as age of the patient Pleuropulmonary blastoma is the most likely diagnosis. I.H.C. report to follow.

Dr. M. J. Shah

29-Jan-2000 to 4-Feb-2000

Constant fever was noted during these days. I also had back pain increasing day by day. We had to wait for the IHC report.

5-Feb-2000

Immunohistochemical Results & Final Diagnosis :
Desmin – Negative, NF – Negative, LCA – Negative
Vimentin – Mod. To strong positivity in 50-60% cells
Myoglobin – weak reaction in margins
Diagnosis : Pleuropulmonary Blastoma.

Dr. M. J. Shah

8-Feb-2000

As, everybody was not ready to accept as true, Papa & Grand Pa went to Tata Memorial Hospital - Bombay with slides & block to have another opinion.

9-Feb-2000 to 14-Feb-2000

Now, these days were more & more dejected. Complications were increasing. I had swelling on right side of my body and face. I couldn’t sit even for one minute. I had intense difficulty in respiration as well. I was put on to oxygen from this morning.· 15-Feb-2000

Microscopical Examination :
This is a round cell sarcoma which is expressing the muscle marker desmin. The tumor cells are negative for Cytokeratin, EMA, Myoglobin, Mic 2, Synaptophysin, LCA and CD3.

There are two major tumors that come into differentiate diagnosis.
1) Embryonal rhabdomyosarcoma.
2) Pleuropulmonary blastoma of which rhabdomyosarcomatous component is biopsied.

Dr. (Ms) Anita M. Borges
M.D., F.R.C.Path.
Tata Memorial Hospital – Bombay.

16-Feb-2000

In the presence of Papa, Mamma & Viru Aunty, I took my last breath at 3.12 AM.

 

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