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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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CASE REPORT
Year : 2012  |  Volume : 17  |  Issue : 2  |  Page : 84-86
 

Proceedings of tumor board meeting


Department of Pediatric Surgery, B.J. Wadia Hospital for Children, Parel, Mumbai, India

Date of Web Publication17-Mar-2012

Correspondence Address:
Sushmita Bhatnagar
56/B, Venus Apartments, Worli Sea Face, Worli, Mumbai - 400 018
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.93976

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   Abstract 

The Tumour Board Meeting was held on August 16, 2011, in the Seminar Hall at B.J. Wadia Hospital for children. The panelists of the meeting were Dr. S. Ranganathan, Pediatric Pathologist from Children's Hospital of Pittsburgh; Dr. Archana Swami, Consultant Pediatric Oncologist at Wadia Children's Hospital; Dr. Sajid Qureshi Onco-surgeon (Pediatric) at Tata Memorial Hospital and Dr. Sushmita Bhatnagar.


Keywords: Solid tumors, children, tumor board meeting, multimodality treatment


How to cite this article:
Bhatnagar S, Madhav M. Proceedings of tumor board meeting. J Indian Assoc Pediatr Surg 2012;17:84-6

How to cite this URL:
Bhatnagar S, Madhav M. Proceedings of tumor board meeting. J Indian Assoc Pediatr Surg [serial online] 2012 [cited 2019 Jun 16];17:84-6. Available from: http://www.jiaps.com/text.asp?2012/17/2/84/93976



   Introduction Top


Appropriate treatment of solid tumors involves a multidisciplinary approach and meticulous selection of the protocol and diligent compliance.


   Case Reports Top


Case 1

A 2-month-old girl presented with increasing abdominal distension and occasional nonbilious vomiting of

1 month duration. She was referred for treatment of large abdominal mass diagnosed on ultrasound elsewhere. On abdominal examination, a large mass occupied almost the entire abdomen with variegated consistency, measuring about 17 × 16 cm. No other significant finding was noted. Ultrasound scan was reviewed and the presence of bilateral large suprarenal masses communicating with each other behind the aorta was reported.

Blood investigations revealed a normal complete blood count, a normal renal function test, serum bilirubin 0.6/0.3 mg%, SGOT (Serum glutamic oxalo-acetic acid) / SGPT (Serum glutamic pyruvic transaminase) 98/70 IU/L, ALP (Alkaline phosphatase) 1594 IU/L, LDH (Lactic Dehydrogenase) 1246 IU/L, uric acid 3.0 mg%, HCG (Human Chorionic Gonadotropin) < 1.2 ng/mL, AFP (Alpha-feto-protein) 252 ng/mL. A computed tomography (CT) scan revealed a large lobulated mass 14.9 × 9.09 × 9.75 cm in size with focal areas of fat, calcification, and fluid containing cystic areas in the retroperitoneum, crossing the midline. The patient was subjected to planned excision of the mass. Intraoperatively, the mass was displacing the entire gut, was adherent to the common bile duct and the portal vein, and was predominantly solid with few cystic areas. The cysts were decompressed by aspiration with a large bore needle. In spite of that, the entire mass could not be excised in toto as it was crossing the midline behind the aorta and inferior vena cava. The mass was cut on the right of the aorta to disconnect both the right and the left halves and the rest of the mass was excised meticulously without any rupture. The specimen weighed 900 g. Histopathology revealed mature teratoma, with no immature or neoplastic elements.

Q: What is the possibility of this mass containing immature elements?

A: Dr. Ranganathan: In such a large tumor, it is not possible to take sections from the entire specimen. However, corroborative evidence, such as the presence of cartilage and bony elements in the tumor, a normal alfa-fetoprotein, and absence of immature elements on histopathology indicate that this tumor is benign. However, teratomas of the peritoneal cavity can show multifocality and close followup would be needed to ensure that there is no local recurrence. Recurrences can be either as teratomas or yolk sac elements.

Q: In view of tumor spillage while separating the right and the left halves, is there a need for chemotherapy?

A: Dr. Swami: Since there is no evidence of malignancy, and the child is just 2 months old, the child should not be given chemotherapy. A close follow up though, is mandatory. If there were immature elements on histopathology, chemotherapy is definitely indicated in the event of tumor spill. Retroperitoneal teratomas with immature elements in infants need not be given chemotherapy unless there is raised alfa-fetoprotein, but becomes mandatory whenever there is tumor spill.

Case 2

A one-and-a-half-year-old male child was admitted with progressive pallor with multiple cervical swellings of 1 month duration. On examination, there was right proptosis, multiple cervical lymphadenopathy but no hepatosplenomegaly. Investigations showed microcytic hypochromic anemia, a right suprarenal mass on abdominal ultrasound. CT scan abdomen/thorax showed neuroblastoma with subpleural pulmonary parenchymal metastases with mediastinal lymphadenopathy. Urinary vinyl mandelic acid (VMA) was 498 mg/g creatinine (normal values being 0-11 mg/g creatinine/24 h). A tru-cut biopsy from the abdominal mass was consistent with neuroblastoma. Positron emission tomography (PET) scan showed right suprarenal uptake as well as bilateral lung, left neck, and multifocal bone marrow involvement. Meta-iodo-benzyl-guanidine (MIBG) scan showed a mass in the right abdomen with extensive bone marrow involvement. Bone scan revealed extensive disease. N-myc gene was not amplified on polymerase chain reaction (PCR). In view of extensive disease, cyclophosphamide, etoposide, carboplatin, cytosine arabinoside (CECA) was planned. After 5 CECA cycles, CT abdomen showed reduction in the primary as well as the secondaries (correct), which corroborated with PET, MIBG, and bone scan. Debulking abdominal surgery was performed followed by 3 CECA cycles.

Surprisingly, histopathology of resected mass showed ganglioneuromatous changes. Contrary to the histopathology report, postchemotherapy (8 CECA cycles), urinary VMA level was 110.3 mg/g creatinine, and PET and MIBG scan showed residual mass at primary site with retroperitoneal lymph nodes and multiple skeletal metastases and new lesions. In view of persisting and progressive lesions, 3 cycles of ifosfamide, carboplatin, etoposide (ICE) were given. Post-ICE, urine VMA was 58.5mg/g creatinine, and PET and MIBG scan showed viable disease in right suprarenal region, right para-aortic region, and in the neck bilaterally. Bone scan showed new metastatic lesions.

In spite of such extensive intervention, the child remained clinically stable and well, contrary to what was evident on investigations. Further treatment in the form of cis-retinoic acid and external beam radiotherapy was planned. Child received 5 cycles of Cis-retinoic acid and rRadio-MIBG therapy, following which urinary VMA remained 64.62 mg/g creatinine, and PET and MIBG scan showed extensive metastatic disease same as before treatment, no new lesions were evident.

Q: How do we explain histopathology of ganglioneuroma?

A: Dr. Ranganathan: Histopathology post-therapy is an unreliable indicator of the type of residual disease. With a positive MIBG and PET scan and raised urinary VMA levels, the tumor has foci of neuroblastoma. Histopathologic changes during or post-therapy does not distinguish between malignant and benign disease. Also the postchemotherapy changes in the primary tumors do not predict the morphology of the tumors at metastatic sites. All post-therapy specimens should be diagnosed as treated neuroblastomas and only the proliferation of anaplastic foci within the tumor may suggest progression or poor behavior.

Q: Would complete excision of primary have helped in better control of the disease?

A: Dr. Sushmita Bhatnagar: Many reports and studies indicate that complete excision of the primary in patients with extensive disease does not change the outcome. The prognosis worldwide in the best of centers with extensive disease is about 30%-40%. Major dissections leading to vascular complications in fact contribute to increased morbidity and mortality in such children.

Q: Do we need both PET scan and MIBG scans for staging in neuroblastoma?

A: Dr. S. Qureshi: MIBG is a more sensitive tool for diagnosis of recurrent/residual disease as compared with PET. PET scan can be utilized in staging and diagnosis of residual disease at the discretion of the treating pediatric oncologist, but is not a formally recommended imaging modality for neuroblastoma. In this patient, the histopathologic diagnosis being ganglioneuroma, and with progressive lesions, which did not corroborate, it was necessary to have an accurate assessment of the lesions. Hence both MIBG and PET scan imaging modalities have been utilized.

Q: What should be the further treatment plan for this child?

A: Dr. Ranganathan: As the child remains well and stable, it might be possible to wait and watch for the progress of the disease, and further treatment should be directed as palliation rather than treatment.

Case 3

A 13-year-old previously healthy male child presented with low-grade undocumented fever with generalized lymphadenopathy of 1 year duration. On physical examination, pallor and generalized lymphadenopathy were the only positive findings. A lymph node biopsy and bone marrow aspiration and biopsy suggested Hodgkin's lymphoma (HL) on histopathology. A baseline PET scan was done and pending immunohistochemistry report started on adriamycin, bleomycin, vinblastin, dacarbazine (ABVD) chemotherapy. Immunohistochemistry available after 1 week showed large atypical cells, which were positive for CD2 (cluster of differentiation 2), negative for CD15, CD30, CD3, activin receptor-like kinase 1, leukocyte common antigen (LCA) expressing PAX-5, negative for multiple myeloma oncogene 1), thus suggestive of NHL T-cell rich, histiocytic-rich B-cell lymphoma.

Since the child showed clinical response with ABVD chemotherapy for HL, a review of the histopathology was asked for, which favored the first diagnosis as Hodgkin's disease mixed cellularity over a T-cell-rich B-cell lymphoma. Six cycles of ABVD chemotherapy were completed. The reassessment PET scan showed poor response to treatment, and a repeat CT-guided biopsy and immunohistochemistry was done from the abdominal lymph node, which ruled out classical Hodgkin's disease or anaplastic large cell lymphoma and favored non-Hodgkin's lymphoma (NHL), large B-cell type. The child was given induction chemotherapy with 2 cycles of doxyfluridine, adriamycin, cyclophosphamide, 1 cycle cyclophosphamide, vincristine, doxorubicin, dexamethasone, consolidation with vinblastin, adriamycin, methotrexate, prednisolone, but with a poor response to treatment. Cervical lymph node biopsy was repeated the third time and now tumor cells expressed CD20, CD-79a, and CD-30 (focal), immunonegative for CD-15, CD-3, background T-cell rich, features suggestive of NHL of diffuse large B-cell phenotype, arising on a background of nodular lymphocyte-predominant lymphoma. The child was thence started with rituximab, cyclophosphamide, doxorubicin (chemical name hydroxydaunorubicin), vincristine (originally named oncovin), prednisolone, completed 4 cycles of chemotherapy till date, reassessment PET scan showing disappearance of FDG (Fludeoxyglucose) uptake in all the sites. The child is clinically stable with no pallor, hepatosplenomegaly, or lymphadenopathy.

Q: What are the differences between Hodgkin's lymphoma and B-cell lymphoma on histopathology?

A: Dr. Ranganathan: Diffuse large B-cell lymphoma (DLBCL) has a different morphology from Hodgkin's disease in that it is made up of diffuse proliferation of large B cells that have the morphology of immunoblasts with prominent nucleoli that show a B-cell phenotype. In contrast, Hodgkin's lymphoma shows a more variegated appearance with a mixed background population of small lymphocytes, eosinophils, and even neutrophils with nodular aggregates of large cells with large vesicular nuclei and prominent nucleoli with scattered classic  Reed-Sternberg cells More Details. Immunostaining plays a very crucial role in differentiating the 2 conditions, especially since the RS cells are CD15 and CD30 positive and lack LCA. In contrast, the cells of large cell lymphoma uniformly express CD20 and/or CD79a as well as bcl6 and lack CD15 and usually CD30. LCA is always positive in these cells. Nodular lymphocyte-predominant Hodgkin's lymphoma shows a lymph node with architecture that is replaced by nodules of B cells without germinal centers and presence of large smudged cells that are CD20 positive and CD15 negative. They are usually associated in the background with progressive transformation of germinal centers.

Q: How does DLBCL usually present?

A: Dr. Sushmita Bhatnagar: The clinical presentation of DLBCL is variable. It usually occurs in old age, but can occur anytime between adolescence and old age. Most commonly children present with painless enlarged lymph nodes. Some of them have B symptoms, such as night sweating, unexplained fever, and weight loss as this child had. The duration of symptoms is dependent on the type of the disease, that is, low grade or high grade. The low-grade DLBCL progresses slowly, whereas the high-grade DLBCL is more rapidly spreading and is more aggressive.


   Conclusions Top


With the rapid advances in the field of Oncology, Tumor Board signifies multidisciplinary management of solid tumors in children in the true sense. All aspects of the tumor, biology, behavior, treatment protocols, and followup plan provide a base for comprehensive management, and thus contribute significantly in the improved final outcome.




 

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