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Year : 2014  |  Volume : 51  |  Issue : 4  |  Page : 510--511

A case of biphasic pulmonary blastoma showing good response to preoperative chemotherapy

P Muthu, A Unnikrishnan, WM Jose, P Keechilat 
 Department of Medical Oncology, Cancer Institute, Amrita Institute of Medical Sciences, Ponekkara, Edappally, Kochi, Kerala, India

Correspondence Address:
Dr. P Muthu
Department of Medical Oncology, Cancer Institute, Amrita Institute of Medical Sciences, Ponekkara, Edappally, Kochi, Kerala
India




How to cite this article:
Muthu P, Unnikrishnan A, Jose W M, Keechilat P. A case of biphasic pulmonary blastoma showing good response to preoperative chemotherapy.Indian J Cancer 2014;51:510-511


How to cite this URL:
Muthu P, Unnikrishnan A, Jose W M, Keechilat P. A case of biphasic pulmonary blastoma showing good response to preoperative chemotherapy. Indian J Cancer [serial online] 2014 [cited 2020 Aug 5 ];51:510-511
Available from: http://www.indianjcancer.com/text.asp?2014/51/4/510/175297


Full Text

Sir,

A 17-year-old male presented to our hospital with complaints of exertional dyspnea of two months and non-productive cough of one month duration. Imaging investigations showed a mass involving the upper and middle lobes of right lung with no mediastinal invasion [Figure 1]. Trucut biopsy was reported as biphasic pulmonary blastoma [Figure 2],[Figure 3],[Figure 4],[Figure 5],[Figure 6],[Figure 7]. The metastatic work-up was negative. The tumor was considered unresectable upfront due to its large size (12 × 10 × 10 cm) with adjacent chest wall infiltration. Hence a preoperative chemotherapy with Cisplatin (at 75 mg/m 2 on day one) and Etoposide (at 100 mg/m 2/day from day one to three) was administered every three weeks. After three such cycles, the patient was reassessed with X-ray [Figure 8] and CT scan, which showed good regression of tumor mass. He then underwent right upper and middle lobectomy followed by adjuvant local irradiation. Patient declined any adjuvant chemotherapy.{Figure 1} {Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}{Figure 7}{Figure 8}

After eight months of follow-up, he presented with local recurrence. Tumorectomy along with excision of segments of fourth and fifth ribs was done. Adjuvant chemotherapy could not be given as the patient refused. After five months, the disease locally recurred for the second time. Since it was bulky and considered unresectable, three more cycles of the same regimen were administered. The tumor was then de-bulked and its residue was irradiated. After being asymptomatic for two months, he was found to have extensive skeletal metastases. He was treated with palliative radiation to the spine and then referred to a local palliative care centre. He had completed two years since diagnosis.

Biphasic pulmonary blastoma (BPB), the most common type of pulmonary blastoma, constitutes about 0.25-0.5% of all primary lung malignancies.[1] Surgery is the mainstay of treatment of BPB irrespective of its stage and extent while chemotherapy and radiotherapy are largely adjuvant modalities.[2] Chemotherapy has been tried with varying success in adjuvant and palliative settings since 1960s and 70s. Review of literature shows that drugs viz. platinum compounds (especially Cisplatin), Etoposide, Adriamycin, Vinca alkaloids, Cyclophosphamide and Actinomycin-D are commonly used.

On the other hand, preoperative chemotherapy for BPB is a relatively newer practice. Buchel reported a case of BPB where the tumor was down-staged with chemotherapy (Cyclophosphamide/Etoposide) before surgery.[3] Similarly, two more patients with advanced tumor mass became operable after preoperative chemotherapy and had good survival period.[4] Recently, in a patient with BPB that had recurred in kidney, laparoscopic radical nephrectomy was done after downsizing the renal mass with Sorafenib.[5]

Cutler et al., on reviewing various adjuvant chemotherapy regimens for the treatment of pulmonary blastoma, have advocated the use of Cisplatin and Etoposide combination.[2] This forms the basis of selection of these two drugs for preoperative chemotherapy in our patient. However, currently there is no higher-level evidence to say which chemotherapeutic drugs are superior to the rest.

References

1Colby TV, Koss MN, Travis WD. Tumors of the lower respiratory tract. In: Bethesda MD, editor. American Registry of Pathology, Armed Forces Institute of Pathology; 1995. p. 395-417.
2Cutler CS, Michel RP, Yassa M, Langleben A. Pulmonary blastoma: Case report of a patient with a 7-year remission and review of chemotherapy experience in the world literature. Cancer 1998;82:462-7.
3Büchel H, Casella R, Müller W, Martinelli G, Cavalli F, Luscieti P. Blastoma of the lung: A rare malignancy. Case report. Helv Chir Acta 1993;60:21-5.
4Zaidi A, Zamvar V, Macbeth F, Gibbs AR, Kulatilake N, Butchart EG. Pulmonary blastoma: Medium-term results from a regional center. Ann Thorac Surg 2002;73:1572-5.
5Mulamalla K, Truskinovsky AM, Dudek AZ. Pulmonary blastoma with renal metastasis responds to sorafenib. J Thorac Oncol 2007;2:344-7.