Clinical Profile:

A 55 year old woman presented with multiple episodes of hemoptysis since a week. She had been having mild chest discomfort for a few months. She is also experiencing headache since past two weeks. There is no known history of Diabetes, Hypertension, Koch’s, Epilepsy or Ischemic heart disease. No history of any addictions

The patient hadaltered sensorium. Vitals are within normal limits and the systemic examination reveals positive Babinski sign on left side. Rest of the CNS examination was normal.

Radiological Investigations:

•The frontal chest radiograph shows a well defined lobulated soft tissue opacity in the right upper and midzone of approx. size 6x8cm. The lateral and superior borders of the lesion are well defined while its inferior border is ill-defined. The superior border is seen one cm below the clavicle. The medial border is seen silhouetting with the right paratracheal stripe and the superior part of the right heart border. However, the lesion is forming an acute angle with the same suggesting its intrathoracic origin and is anterior or has an anterior extension. There is no cavitation or calcification within the lesion.Mild thickening of the horizontal fissure is seen.

A diagnosis of an intraparenchymal pulmonary mass, likely malignant (size>3cm) in the anterior part of right upper zone was made.

An HRCT and CECT chest was advised for further evaluation

Multiplanar reconstructed images of the HRCT chest with pulmonary angiographic and post contrast phase shows a well defined lobulated heterogeneously enhancing mass lesion with cystic components and calcific foci within in the right upper lobe of approx. size 6.4x8cm. The mass is extending along the major fissure and causing its nodular thickening with extension into the superior segment of the right lower lobe. The right upper lobe bronchus supplying is not seen separately from the lesion suggesting either its compression or invasion which is causing atelectasis of the upper lobe.

The mass is invading the pulmonary veins causing its partial occlusion, encroaching upto the opening of pulmonary veins into the left atrium.

PET CT was advised.

•A PET-CT shows increased metabolic activity within the mass lesion and its extension along the pulmonary vein and left atrium.

•A provisional diagnosis of non-small cell carcinoma of the lung was made. A biopsy of the lesion was advised. Also, in view of the CNS complaints an MRI of the brain was advised.

•MRI of the brain shows a T2/FLAIR hyperintense lesion of size 1.2x0.9cm in the right frontal lobe with significant lamellated post contrast enhancement predominantly in the centre with isointense borders likely due to reactive edema.

•The CT brain reveals a hyperdense lesion in the right frontoparietal lobes with significant surrounding edema and mass effect in the form of effacement of adjacent sulci, frontal horn and body of right lateral ventricle and midline shift suggestive of hemorrhagic metastasis.

•CT guided biopsy of the pulmonary mass lesion was performed

Pathological findings:

There are predominantly spindle cells and focal pleomorphic areas. SMA (smooth muscle actin) and EMA (epithelial membrane antigen) were both positive.

Final diagnosis : Intimal Sarcoma of lung with brain metastasis was made.

Differential diagnosis :

•The aforementioned case basically comprises of a lung mass with a pulmonary vein thrombus and brain metastasis.

•The most frequent malignant cause of pulmonary vein thrombus (PVT) is a primary lung neoplasm, especially bronchogenic carcinoma . However, PVT can also occur following a metastatic cancer such as small cell lung cancer, metastatic sarcoma and liposar­coma (3) or an Intimal sarcoma of pulmonary vein itself as present in this case.

•Small cell lung cancer (SCLC)- small centrally located tumors with significant hilar and mediastinal lymphadenopathy. A mass in or adjacent to the hilum is characteristic of SCLC and the tumour may well show mediastinal invasion. Pleural effusions occur in upto 40% of the patients. Multiple metastasis to liver, adrenals, bone marrow and brain can be found.

•Adenocarcinomas usually present as a peripheral solitary pulmonary nodule with an irregular or spiculated margins owing to lepidic pattern of spread. The attenuation can be ground glass or that of a soft tissue. Hilar and mediastinal involvement is seen in half of the cases. A variant, Lymphangitis carcinomatosis on imaging appears as thickened interlobular septa or multiple small pulmonary nodules

•Large or giant cell carcinoma is a poorly differentiated non small cell carcinoma (NSCLC) and is diagnosed histologically after exclusion of adeno-carcinomatous or squamous differentiation . They are peripherally located masses with ill defined margins growing more than 7cm in diameter. It commonly spreads to local lymph nodes while cavitation is rare. (4)

•A Pancoast tumor is a tumor of the superior pulmonary sulcus characterized by Horner's syndrome and pain due to invasion of the brachial plexus.

•Approximately 10 percent of newly diagnosed patients with advanced non-small cell lung cancer (NSCLC) have brain metastases. Of patients with brain metastases, lung cancer is the primary tumor in 40 to 50 percent of cases. (5,6)

•Hence it may be concluded upon that brain imaging is advisable at the time of diagnosis of lung cancer to look for asymptomatic metastasis. The presence of a brain metastasis would change the TNM staging and hence the treatment too.

•On imaging, the metastasis are located at the gray–white junction and at border zones between major arterial vascular territories. Certain tumors have a predisposition to produce hemorrhagic metastasis which includes lung (NSCLC), breast, melanoma, RCC and thyroid malignancies. Iodinated contrast enhancement is vital to the detection of metastases on CT, and brain metastases may demonstrate ring, nodular, or solid enhancement. A point to be noted is that the metastasis can be solitary or multiple. (7)

Treatment Options

•Lobectomy is generally not possible if there is:

1.Transfissural extension

2.Invasion of main and segmental bronchi

3.Pulmonary vascular invasion.

•SCLC- Usually progressed by the time diagnosis is made. Chemoradiotherapy is the primary treatment modality

NSCLC- Stage I or II- Complete surgical resection +/- adjuvant chemotherapy. Stage III- neoadjuvant and adjuvant chemotherapy with surgery. Stage IV- palliative care or

References:

1.Xu, Rui MMa; Zhao, Yixuan PhDa; Xu, Xiaosen MMb; Liu, Shuang MMa; Hu, Chenyu MMa; Lv, Dongmei MD, PhDa; Wu, Huiying MDa,∗ Pulmonary intimal sarcoma involving the pulmonary valve and right ventricular outflow tract, Medicine: January 2020 - Volume 99 - Issue 3 - p e18813

2.Barmpas A, Giannakidis D, Koulouris C, Mantalobas S, Pavlidis E, Koimtzis G, et al. Intimal sarcoma of the pulmonary artery, a diagnostic enigma. AME Case Reports; Vol 3 (August 2019): AME Case Reports [Internet]. 2019 [cited 2019 Jan 1]

3.Chaaya G, Vishnubhotla P. Pulmonary Vein Thrombosis: A Recent Systematic Review. Cureus [Internet]. [cited 2020 Sep 22];9(1).

4.CT and MRI of the Whole Body-John R. Haaga, Daniel T. Boll

5.Barnholtz-Sloan JS, Sloan AE, Davis FG, Vigneau FD, Lai P, Sawaya RE. Incidence proportions of brain metastases in patients diagnosed (1973 to 2001) in the Metropolitan Detroit Cancer Surveillance System. J Clin Oncol. 2004 Jul 15;22(14):2865–72.

6.Incidence of brain metastases in a cohort of patients with carcinoma of the breast, colon, kidney, and lung and melanoma - PubMed [Internet]. [cited 2020 Sep 22].

7.Fink KR, Fink JR. Imaging of brain metastases. Surg Neurol Int. 2013 May 2;4(Suppl 4):S209–19.

8.www.uptodate.com/contents/overview-of-the-initial-treatment-and-prognosis-of-lung-cancer?search=lung%20cancer&source=search_result&selectedTitle=3~150&usage_type=default&display_rank=3