Journal of The Association of Physicians of India ■ Vol. 63 ■ November 2015 69

Atypical Bronchial Carcinoid Masquerading as Bronchial AsthmaV Rajendran1, Iqbal2, Vinod Kumar3

1Associate Professor of Medicine, 3Post Graduate, Department of Medicine, KAPV Medical College and Mahatma Gandhi Memorial Government General Hospital (MGMGH), Trichirapalli; 2Post Graduate, Department of Medicine, Thanjavur Medical college, Thanjavur, Tamil NaduReceived: 03.02.20914; Revised: 22.08.2014; Accepted: 23.09.2014

Abstract A case study of 35-year-old woman with persistent breathlessness and wheezing that had been unsuccessfully treated with inhaled beta 2-agonists and steroids for about two years. Patient developed dry cough and haemoptysis, so investigated further. Spirometry demonstrated a restrictive pattern. Chest CT demonstrated well defined hyperdense lesion in right middle lobe. Biopsy taken from the mass during bronchoscopy demonstrated the picture of atypical bronchial carcinoid. In this case, due to the lack of awareness, diagnosis of carcinoid was delayed by two years.

Introduction

Carcinoid tumors are malignant neuro endocrine tumors arising

from kulchitsky cell. Most common site of carcinoid tumors is GI tract (64%) next is being respiratory tract (28%). Bronchial carcinoids accounts for 1-2 % of all lung malignancies. Male and female are equally affected with mean age of presentation is 40 years.

It is a very slow growing tumor hence called “cancer in slow motion”. Bronchial carcinoids are frequently discovered as a lesion on a chest radiograph and 31% of the patients are asymptomatic. Based on microscopic appearance they are called typical carcinoid when less than two mitosis present after ten high power field examination and without areas of necros is . Atypica l carc inoids are diagnosed when more than ten mitosis detected after ten high power field examinations or by the presence of necrosis. WHO classification includes f o u r g e n e r a l c a t e g o r i e s : t y p i c a l carcinoid, atypical carcinoid, large

cell neuroendocrine carcinoma, small cell carcinoma. Typical and atypical categories are not related to tobacco use but not the other two. Prognosis is excellent for typical carcinoids and poor for small cell carcinoma.

Case Report

A 35- year-old female admitted with breathlessness and wheezing for about two years, cough and 6 to 7 episodes of haemoptysis for two months. This wheezing had no correlation with physical exertion. She was treated as a case of Bronchial asthma with bronchodilators and steroids, both oral and inhaled.

On Physical examination patient was found to be moderately built and nourished, blood pressure was 130/80 mm Hg, Pulse rate was 86 per minute, Respiratory rate was 18 per minute. Breath sounds and vocal resonance were decreased over right axillary and infra-axillary areas. Bilateral wheeze

noted. Patient’s skin showed suggestion of flushing though she was dark.

H e m a t o l o g i c a l i n ve s t i g a t i o n s revealed haemoglobin 9.2 gms/dl, total leucocyte count 12000 (P-66, L-30, E-4), platelet count 2 lac/cu.mm, ESR 8 mm at the end of one hour. Urea 34 mgs/dl, creatinine 0.9 mgs/dl, random blood sugar 112 mgs/dl.

Chest x-ray demonstrated a well defined opacity in the right lower zone (Figure 1). CT chest revealed a mass lesion involving Right middle lobe (Figure 2 and 3). Staging work up done. No evidence of distant metastasis detected.

Pulmonary function tests revealed restr ic t ive pattern. Bronchoscopy revealed right sided endobronchial g r o w t h . B i o p s i e d s p e c i m e n demonstrated atypical carcinoid tumor by histopathological examination.

Patient underwent middle lobectomy ( F i g u r e s 4 ) . H i s t o p a t h o l o g i c a l examination of resected specimen confirmed atypical carcinoid tumor (Figure 5).

Discussion

Bronchial carcinoids accounts for 1-2% of primary lung tumors may be located centrally as endobronchial carcinoid and present asymptomatically or produce wheeze, haemoptysis , p o s t - o b s t r u c t i v e p n e u m o n i t i s . Peripherally located carcinoids occurs a s y m p t o m a t i c a l l y o r s c a r r i n g i n

Fig. 1: Preoperative CXR

Fig. 2: CT showing tumor Fig. 3: CT showing tumor

Journal of The Association of Physicians of India ■ Vol. 63 ■ November 201570

nature. Carcinoids may present as multiple tumorlets producing airway fibrosis leading to severe obstructive lung disease. All carcinoid tumors do not cause carcinoid syndrome. T h e r e a s o n i s e ve n t h o u g h h i g h level of neuropeptide and amine are synthesized they may not be released in enough high quantity or due to defective chemical nature. Clinical features of carcinoid syndrome includes flushing, watery diarrhea, wheeze, asthma-like symptom, pellagra-like skin lesion and retroperitoneal fibrosis, Peyronie’s disease commonly seen in those with liver metastasis or tumors outside the gastrointestinal tract like ovarian or lung carcinoids.

In chest X-ray bronchial carcinoids manifests as solitary pulmonary nodule

or calcified nodule in 40%, infiltrations in 60% of cases. CT and somatostatin r e c e p t o r s c i n t i g r a p h y l o c a l i z e s metastatic deposits in liver. MRI shows high signal intensity in T2 weighted images. Serum chromogranin-A level correlates with tumor bulk. Plasma NSE, platelet serotonin and urinary 5HIAA (typical carcinoid), 5HTP and 5HT (atypical carcinoid) supports the diagnosis.

Typical carcinoids are treated by surgical resection of tumour, atypical carcinoids require lobectomy and lymphnodal dissection. Cisplatin and etoposide based chemotherapy used in unresectable tumors. Liver metastasis treated by chemo embolisation using 5-flurouracil, doxorubicin and cisplatin. Carcinoid syndrome is treated by

octreotide or lantreotide and interferon alpha.

Conclusion

This case is a good example of masquerading of a rare disease as a common il lness. Extended clinical d i a g n o s i s , i n c l u d i n g c o m p u t e d t o m o g r a p h y a n d b r o n c h o s c o p y , should be considered in all cases of bronchial asthma or chronic obstructive pulmonary disease which do not respond to standard treatment.

References1. Santra A, Dutta P, Pothal S, Manjhi R. Misdiagnosed case of

bronchial carcinoid presenting with refractory dyspnoea and wheeze: a rare case report and review of literature. Malays J Med Sci 2013; 20:78-82.

2. Robby BB, Drehner D, Sidman JD. Pediatric tracheal and endobronchial tumors: an institutional experience. Arch Otolaryngol Head Neck Surg 2011; 137:925-9.

3. Andersen JB, Mortensen J, Damgaard K, Skov M, Sparup J, Petersen BL, Rechnitzer C, Borgwardt L. Fourteen-year- old girl with endobronchial carcinoid tumour presenting with asthma and lobar emphysema. Clin Respir J 2010; 4:120-4.

4. Bolukbas S, Eberlein M, Schirren J. A 30-year-old woman with only right sided asthma? Thorac Cardiovasc Surg 2010; 58:120-2.

5. Steinfort DP, Finlay M, Irving LB. Diagnosis of peripheral pulmonary carcinoid tumor using endobronchial ultrasound. Ann Thorac Med 2008; 3:146-8.

6. Dipaolo F, Stull MA. Bronchial carcinoid presenting as refractory asthma. Am Fam Physician 1993; 48:785-9.

Fig. 4: Gross appearance Fig. 5: Microscopic appearance