|Year : 2018 | Volume
| Issue : 3 | Page : 289-292
A rare variety of pulmonary blastoma: A case report and review of literature
L Purnima Devi1, Umesh Das2, Rintu Marak2, Jyoti Prasad Kalita3, Yookarin Khongla4, Baphirayne Wankhar5, Ibanylla Meggsi Pala Rymbai2
1 Department of Radiotherapy, NEIGRIHMS, Shillong, Meghalaya, India
2 Department of Medical Oncology, NEIGRIHMS, Shillong, Meghalaya, India
3 Department of CTVS, NEIGRIHMS, Shillong, Meghalaya, India
4 Department of Pathology, NEIGRIHMS, Shillong, Meghalaya, India
5 Department of Radiodiagnosis, NEIGRIHMS, Shillong, Meghalaya, India
|Date of Web Publication||25-Sep-2018|
Dr. Umesh Das
Department of Medical Oncology, NEIGRIHMS, Shillong, Meghalaya
Source of Support: None, Conflict of Interest: None
Pulmonary blastoma (PB) is a very rare primary lung neoplasm. Morphologically, they mimic fetal lung tissue before 4 months gestation. As monophasic variety is extremely rare, we have reported here a case of monophasic PB in a young adult patient who came with cough and hemoptysis for 4 months. On computed tomography scan of the chest, findings showed left upper lobe mass with few specks of calcification within the lesion. The patient underwent left upper lobectomy. The diagnosis was confirmed through histopathological examination with immunohistochemical evaluation. Because of its extreme rarity, we report this case with special interest to help and improve in faster diagnosis and management of the patients diagnosed with PB in future.
Keywords: Lung neoplasm, monophasic pulmonary blastoma, pulmonary blastoma
|How to cite this article:|
Devi L P, Das U, Marak R, Kalita JP, Khongla Y, Wankhar B, Pala Rymbai IM. A rare variety of pulmonary blastoma: A case report and review of literature. Indian J Health Sci Biomed Res 2018;11:289-92
|How to cite this URL:|
Devi L P, Das U, Marak R, Kalita JP, Khongla Y, Wankhar B, Pala Rymbai IM. A rare variety of pulmonary blastoma: A case report and review of literature. Indian J Health Sci Biomed Res [serial online] 2018 [cited 2018 Dec 15];11:289-92. Available from: http://www.ijournalhs.org/text.asp?2018/11/3/289/242049
| Introduction|| |
Pulmonary blastoma (PB) is a very rare pulmonary neoplasm, accounting for 0.25%–0.5% of primitive malignant tumors of the lung. First described by Barnard  and later on by Koss et al., further subdivided this entity into three categories based on tissue component: (i) monophasic PB, known as the well-differentiated fetal adenocarcinoma (WDFA) having epithelial malignant component only; (ii) the classic biphasic pulmonary blastoma (CBPB) characterized by both epithelial and mesenchymal malignant components; and (iii) pleuropulmonary blastoma, that is a childhood tumor with features of mesenchymal malignant components only and currently considered as a separate entity. Monophasic PB is extremely rare having only epithelial malignant tissue component. In the World Health Organization classification, PB is included under the category of carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements. PB is observed in the fourth decade of life with a mean age of occurrence in adults being 43 years and shows a strong female preponderance. Because of its extreme rarity, there have been no controlled clinical trials investigating treatment regimens for fetal adenocarcinoma, and as a result, there are no guidelines for management. Herein, we report a 19-year-old male affected with monophasic variety of PB with special interest to help and guide physicians in faster diagnosis and better management of the patients diagnosed with PB in future.
| Case Report|| |
A 19-year-old male, nonsmoker presented with cough and hemoptysis for 4 months for which he has taken anti-tuberculous treatment for 4 months. There was no history of fever, night sweats, wheeze, chest pain, or weight loss. Family history was unremarkable. On physical examination, he was well built with slight pallor without any lymphadenopathy. On examination, bilateral lungs were clear; trachea was central. There was no evidence of pleural effusion. Baseline investigations of the patient were nonsignificant except mild thrombocytopenia. Chest X-ray showed homogenous opacity (L) upper zone. Computed tomography (CT) scan thorax showed a well-defined soft-tissue density rounded lesion (4.79 cm × 3.83 cm × 4.97 cm) in the postsegment of the left upper lobe. Few specks of calcification were noted within the lesion. Multiple calcified nodules in the bilateral lung fields predominantly in upper lobe [Figure 1], [Figure 2], [Figure 3]. Given a possible neoplastic lesion on clinical and radiological assessment, a fine-needle aspiration cytology (FNAC) of the lesion was performed that revealed blastemal cell in favor of pulmonary of the PB. His electrocardiogram and ultrasound abdomen were reported as normal. The patient underwent left upper lobectomy through left postero-lateral thoracotomy. The histopathology of the specimen was suggestive of PB, monophasic epithelial variant with well-differentiated fetal type adenocarcinoma with lymphovascular invasion and infiltration of pleura and tumor cells are PAS-positive and diastase sensitive [Figure 3] and [Figure 4]. Postoperative course was uneventful. The patient was given six cycles of ifosfamide, etoposide and carboplatin-based adjuvant chemotherapy and there was no evidence of recurrence or distant metastasis in the 8 months of follow-up.
|Figure 1: Contrast enhanced computed tomography of the thorax in mediastinal window reveals a rounded soft tissue density lesion in the posterior segment of left upper lobe measuring 4.9 × 4.7 × 3.8 cm. Few specks of calcification noted within the lesion|
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|Figure 2: Coronal reformation computed tomography in lung window shows that the mass lesion has a well defined margin with no spiculation|
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|Figure 3: H and E Stain, ×10 - well-differentiated tubular glands lined by fetal-like epithelium with subnuclear and supranuclear cytoplasmic vacuolation which was PAS positive diastase sensitive indicating glycogen|
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| Discussion|| |
PB is a rare tumor with distinctive biological behavior. In 1952, it was first described by Barnard  as an “embryoma.” The term “blastoma” was introduced later in 1961 by Spencer who described the tumor origin from a pleuripotential PB analogous to Wilm's tumor that appears to share the same histological features. WDFA is a subtype of PB, which was first described by Kradin et al., in 1982. Currently, PB is regarded as a type of sarcomatoid carcinomas. It is a biphasic tumor containing fetal adenocarcinoma (typically of low grade) and primitive mesenchymal stroma.,
The etiology is still uncertain, but there is strong evidence in the literature showing the correlation between cigarette smoking and PB. The types of p53 mutations are also similar to the p53 mutations found in other lung cancers. Considerable immunoreactivity to tumor cell for alpha-fetoprotein (AFP) was reported in the literature, but unfortunately, AFP levels were not measured in our patient.
The average age was 39 years for the occurrence of CBPB as reported by Van Loo et al. CBPB can occur at any age from birth to 70 years, and of which 80% of CBPB occurs in an adult individual. CBPB is common among the smokers, and it has slight male preponderance, but our patient is a young adult with no history of smoking.
Most patients are asymptomatic or present with cough, bloody sputum, dyspnea, chest, or back pain which is sometimes accompanied by intrathoracic bleeding. In our case, cough and hemoptysis were the main symptoms. In our case, initially, he was misdiagnosed with pulmonary tuberculosis and was on antitubercular treatment but without any response.
WDFA is defined by a monophasic carcinoma solely consisting of malignant epithelial components (squamoid morules) with subnuclear and supranuclear vacuoles, and a benign-appearing mesenchymal stroma.,,
Immunohistochemistry plays a vital role in the diagnosis of PB and expression of cytokeratin and vimentin is seen in the blastomatous component, the epithelial elements in blastomas react positively for keratin, carcinoembryonic antigen, epithelial membrane antigen, and milk fat globulin. Nakatani et al.'s 19 β-catenin overexpression and aberrant nuclear/cytoplasmic localization of β-catenin have a potential diagnostic role of WDFA. Yamazaki K18 TTF-1, GATA-6 TTF-1, and GATA-6 may partially and incompletely simulate fetal lung epithelial morphogenesis.
Because of the rarity of this tumor, treatment remains controversial, and the efficacy of adjuvant chemotherapy and radiotherapy has not been well established.
Surgery is the mainstay of treatment of PB irrespective of its stage and extent while chemotherapy and radiotherapy are largely adjuvant modalities. Chemotherapy has been tried with varying success in adjuvant and palliative settings since the 1960s and 1970s. Postoperative chemotherapy or radiotherapy is selectively used for patients with advanced lung cancers, but the potential benefits in WDFA are unclear.
Review of literature shows that drugs, namely, platinum compounds (especially cisplatin), etoposide, adriamycin, vinca alkaloids, cyclophosphamide, and actinomycin-D are commonly used.
Our patient had undergone surgical resection followed by six cycles adjuvant chemotherapy ICE (ifosfamide, cisplatin, and etoposide) We choose this protocol because of the sensibility of fetal tumors to the combination based on etoposide and cisplatin-like nephroblastoma.
In our case, the decision to start adjuvant treatment was based on the incomplete surgery, as lymph node dissection was not done but due to lack of established evidence of the role of radiotherapy in controlling local recurrence, the patient did not receive adjuvant radiotherapy.
The prognosis of PB is poor, although prognosis of WDFA is better than biphasic.
Koss et al. mentioned that the median survival was approximately 1 year. The factors that indicate poor prognosis are tumor recurrence, metastasis at initial presentation, tumor size over 5 cm and lymph node, and distant organ metastasis. Liver, central nervous system, and bones are the most frequent location of distant metastases.
Koss et al. reported 28 cases of WDFA, with long follow-up of 95 months, and 5-year survival of 81%.
Our patient is on ongoing 3 monthly follow-up with complete remission. Reevaluation CT scans will be done, and the patient is advised to seek immediate medical care in case of appearance of new symptoms.
| Conclusion|| |
Monophasic PB is a rare subtype of PB with a better prognosis. With the challenging nature of its histology, it is difficult to diagnose this disease with a small biopsy specimen. Surgical resection is the treatment of choice for resectable disease. Further studies with more patients are needed to determine the characteristics of the tumor and optimal management.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]