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Year : 2015  |  Volume : 52  |  Issue : 4  |  Page : 555--556

“Low-grade adenocarcinoma of fetal lung type: In an elderly non-smoker female” with aberrant β-catenin expression

S Pasricha, JS Gandhi, A Sharma, Anurag Mehta 
 Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5, Rohini, New Delhi, India

Correspondence Address:
S Pasricha
Department of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5, Rohini, New Delhi
India




How to cite this article:
Pasricha S, Gandhi J S, Sharma A, Mehta A. “Low-grade adenocarcinoma of fetal lung type: In an elderly non-smoker female” with aberrant β-catenin expression.Indian J Cancer 2015;52:555-556


How to cite this URL:
Pasricha S, Gandhi J S, Sharma A, Mehta A. “Low-grade adenocarcinoma of fetal lung type: In an elderly non-smoker female” with aberrant β-catenin expression. Indian J Cancer [serial online] 2015 [cited 2020 Jul 5 ];52:555-556
Available from: http://www.indianjcancer.com/text.asp?2015/52/4/555/178439


Full Text

Sir,

Low-grade adenocarcinoma of fetal lung type (L-FLAC)/well-differentiated fetal adenocarcinoma (WDFA) is a rare but distinct diagnostic entity comprising of 0.25% - 0.5% all primary lung tumors. The peak incidence is in fourth decade, with a mild female predominance, with fair prognosis and low death rate.[1],[2],[3] We report a case of L-FLAC.

A 58-years-old hypertensive non-smoker female presented with complaints of hemoptysis for 3 weeks duration. Routine lab investigations (hematological and biochemistry parameters) were normal. Computed tomography (CT)-chest revealed a lobulated soft tissue density mass lesion involving the upper lobe of right lung, measuring 51 × 41 mm [Figure 1]. No significant intra-thoracic lymphadenopathy or pleural effusion evident. Subsequently, whole body positron emission tomography (PET)-CT findings were consistent with the known mass lesion and were suggestive of malignancy. No other metabolically active foci or mass lesion evident. CT-guided FNAC was hemorrhagic and inconclusive. Patient underwent right lobectomy. Grossly, specimen measured 13 × 9 × 2 cm with peripheral nodular mass measuring 4 cm in maximum dimension. Overlying visceral pleura and bronchial cut margin were grossly free.{Figure 1}

Microscopic examination showed a fairly circumscribed neoplasm comprising of complex glandular architecture with tubular, papillary, and cribriform pattern admixed with scant spindle cell stroma. The glandular epithelium was columnar having ovoid bland nuclei with minimal stratification. Supranuclear and subnuclear clearing of cytoplasm was seen at places resembling secretory endometrium [Figure 2]. Mitotic activity was sparse. Solid morule formation with focal squamoid differentiation was evident. No sarcomatous area was seen. Tumor cells were positive for keratin (AE1/AE3 antibody), keratin 7, epithelial membrane antigen, beta(β)-catenin, chromogranin A, synaptophysin, and neural cell adhesion molecule (N-CAM) [Figure 3] and were negative for thyroid transcription factor (TTF-1), p63, p53, CK5, and alpha-feto protein. Stromal cells were positive for vimentin. Morules were strongly positive for Keratin, β-catenin, and weakly for chromogranin A. β-catenin expression [Figure 4]a] was aberrant nuclear/cytoplasmic (N/C). This expression pattern of β-catenin resembles that of a developing fetal lung in early weeks of gestation. At later stages of gestation, the peripheral branching airway epithelium show only membranous positivity, which was evident in the non-neoplastic lung parenchyma [Figure 4]b] in presented case.[2] Hence, a final diagnosis of L-FLAC, stage IB was rendered. This tumor characteristically occurs in fourth decade with majority (80%) associated with history of smoking. However, the presented case occurred in an elderly non-smoker female. Usually high grade (H)-FLAC is predominantly seen in the elderly.[2],[4]{Figure 2}{Figure 3}{Figure 4}

Differential diagnosis comprises of papillary adenocarcinoma of lung, H-FLAC, and metastatic adenocarcinoma from endometrium. Morphological features with characteristic IHC findings and whole body PET-CT scan helped to rule out the differentials and metastatic origin. Nakatani et al.[2] have evaluated 11 cases of L-FLAC, all of which showed aberrant N/C expression of β-catenin, whereas majority of H-FLAC and conventional adenocarcinoma (CAC) of lung showed membranous expression. Their study suggested that up-regulating disturbances in the Wnt signaling pathway, including mutation of the β-catenin gene, underlie the tumorogeneis of L-FLAC while H-FLAC and CAC of lung appears to have different oncogenic pathway.

Hence, it is important to recognize this rare entity with favorable prognosis and low death rate with complete surgical excision being preferred modality of treatment.

 Acknowledgements



Dr. Damandeep Kaur, Senior Resident. Department Of Pathology, Rajiv Gandhi Cancer Institute and Research Centre, Sector 5, Rohini, New Delhi- 110085.

References

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2Nagashima Y, Shimoyama K, Nakamura N, Sano J, Ogawa N, Shibagaki T, et al. Aberrant nuclear localization and gene mutation of beta-catenin in low-grade adenocarcinoma of fetal lung type: Up-regulation of the Wnt signaling pathway may be a common denominator for the development of tumors that form morules. Mod Pathol 2002;15:617-24.
3Vaideeswar P, Agarwal AP. Foetal adenocarcinoma of the lung. J Postgrad Med 2004;50:75-6.
4Nakatani Y, Kitamura H, Inayama Y, Kamijo S, Nagashima Y, Shimoyama K, et al. Pulmonary adenocarcinomas of the fetal lung type. A clinicopathologic study indicating differences in histology, epidemiology and natural history of low-grade and high-grade forms. Am J Surg Pathol 1998;22:399-411.