Primary amyloidosis of the trachea S. G.

DEODHARE*

and G. DASGUPTAf (Miraj, India)

PRIMARY amyloidosis involving the tracheo-bronchial tree is rare. In a review of literature, Gottlieb and Gold (1972) recorded 23 cases of tracheobronchial amyloidosis. Primary amyloidosis exclusively involving the trachea is still rare. There are only three reports of solitary amyloidosis of the trachea in the literature (Kraus (1886) cited by Antunes and Vieira Da Laz, 1969; Hollinger, 1950; McAlpine et al., 1963). The case reported herein brings the total to four. Our case manifested clinically as a tumour-like mass in the trachea.

Case report

R. S. P., a 40-year-old female, was admitted to the Wanless Hospital on 18.2.74 with gradually progressive breathlessness of two years' duration. She also complained of dry cough for the same period. She had received anti-asthma treatment from time to time without much relief. On examination: A moderately built and nourished lady who spoke with a slightly muffled voice, had a respiratory stridor of moderate degree. There was no cyanosis at the time of examination. On indirect laryngoscopy a pink, irregular mass was seen about 1 cm. below the vocal cords. No significant lymphadenopathy was noticed. A provisional diagnosis of subglottic carcinoma was made and the patient was sent for admission. While the patient was waiting in front of the admission office, she was suddenly bitten by some bees on the face and neck and her dyspnoea became acute and made her almost asphyxiate. As a result an emergency tracheostomy had to be performed in the Casualty department between the 2nd and 3rd tracheal rings. At the time of the tracheostomy, the same growth, as seen by indirect laryngoscopy, was encountered in the lumen of the trachea which made the passage of the tube difficult. At this point the diagnosis was changed to a tracheal tumour. In spite of the tracheostomy the patient was having dyspnoea to some extent which was relieved by passing a long tracheostomy tube. Direct laryngoscopic examination revealed an irregular pinkish mass about 1 cm. below the vocal cords on the anterior and lateral walls. A biopsy from the mass was obtained for histopathological examination. Pathological findings Gross findings: The gross specimen consisted of many irregular pink, soft and pliable pieces, varying in size from 0-2x0-3 cm, to i x 1-5 cm. with a waxy appearance. *Professor and Head of the Department of Pathology, Wanless Hospital and Miraj Medical College, Miraj, Maharashtra, INDIA. t Reader and Head of the Department of Otolaryngology, Wanless Hospital and Miraj, Medical College, Miraj, Maharashtra, INDIA.

645

S. G. Deodhare and G. Dasgupta Light microscopy: Formalin fixed, paraffin embedded sections of tissue stained with haematoxylin and eosin, Crystal violet, Congo red and Thioflavine —T were examined. When stained with haematoxylin and eosin, the tissue showed a pseudostratined columnar epithelial lining (Fig. i). Irregular, conglomerated masses of amorphous, acellular, homogenous eosinophilic material were seen in the

FIG. I.

Photomicrograph showing amyloid deposits and the intact epithelium (H & E X ioo)

submucosa (Fig. 2). Embedded in the material were seen atrophic mucous glands. The blood vessels did not show any abnormality. The stroma exhibited partial fibrosis and mild lymphocytic infiltration. Multinucleated giant cells and foci of calcification were not seen. There was a transition to squamous epithelium in one area. At one place, amyloid material was fused with a piece of cartilage. Section stained with Crystal violet showed an intense positive metachromatic reaction. The Congo red stain gave a bright red colour and when these sections were observed under the polarised light, a definite green birefringence was obtained. Sections were stained with Thiflavine—T by the method of Burns et al. (1967). These sections were examined using HBO 200 Zeiss pressure mercury lamp as the ultra violet light source. Amyloid exhibited intense green to blue fluorescence against the dark background. The patient was subjected to detailed laboratory investigations to rule out associated chronic disease and multiple myeloma. Haematological values were within normal limits. Bone marrow was normoblastic. Examination of urine did not show albumin or Bence Jones protein. Biochemical studies for serum 646

Clinical records

FIG. 2. Biopsy of trachea taken at bronchoscopy showing irregular conglomerated masses of amyloid. (H & E X40)

proteins, serum bilirubin, S.G.O.T. and serum alkaline phosphatase were within normal limits. Immunoelectrophoretic study of the patient's serum disclosed no abnormality. Electrocardiogram was normal. Chest X-ray films were normal. Bronchoscopy through the tracheostomy showed that the lesion did not extend up to the bronchus. As it was evident from the investigations that the disease was confined to the trachea, excision of the growth with split thickness skin graft was planned. On 1.3.74, under general anaesthesia through a cuffed tracheostomy tube, a midline incision was made from the thyroid notch to the upper border of the tracheostome and again from the lower border of the tracheostome to the suprasternal notch, so as to expose the full length of the trachea in the neck. The upper three rings of the trachea were split vertically and the splitting in the manner was continued from the lower border of the tracheostome up to the 7th ring. A window on the anterior wall of trachea was made in the region of the first and second rings to have a better look inside the trachea and subglottic region (Fig. 3). A pinkish granular mass was found to be occupying the whole of the anterior and lateral walls of the trachea from the first ring. The lower extent was found to be up to the 7th ring after deflating the cuffed tube momentarily. The subglottic region was examined with a small laryngeal mirror and found to be free. With a fine SMR dissector the mass was easily separated from the walls of the trachea with minimal bleeding. Clearance at the lower part was obtained after taking out the cuffed tube for about a minute with the permission of the anaesthetist. A split thickness skin graft was obtained from the right thigh and it was mounted on a glove finger which was kept expanded by rubber 647

S. G. Deodhare and G. Dasgupta

FIG. 3. Schematic diagram of the operation—A—1st tracheal ring; B—7th tracheal ring; C— Emergency tracheostomy opening; D—Tracheostomy op?ning at the end of operation; E—Mid-line incision over the trachea; F—Fenestrated pait of the trachea; G—Flap of the fenestrated part.

foam packing. The graft was deficient posteriorly. The glove finger was secured to the neck skin by silk stitches at the upper and lower ends and the tracheostome was changed into the 7th ring. The edges of the tracheal incision were apposed to each other without any stitches. The skin incision was closed with interrupted silk stitches. A metal tracheostomy tube was kept for 10 days post-operatively. The stitches and the glove finger were removed on the 5th post-operative day. Gradual detubation was started from the n t h day and by the 15th day the patient was without a tube and breathing freely. Post-operative follow up for the last 5 months did not reveal any recurrence and the patient is symptoms free. Discussion The present case is a case of primary amyloidosis since the patient showed no clinical evidence of chonic disease associated with secondary amyloidosis. Bone marrow, urine, and serum protein electrophoresis studies were negative for multiple myeloma, which is frequently associated with amyloidosis. Kraus (1886) reported the first case of amyloidosis of the trachea at autopsy. The second case was reported by Holinger et al. (1950). The diagnosis of this case was made by bronchoscopy. The third case was reported by McAlpine et al. (1963). The Table shows the clinical features of the reported cases of amyloid648

Year

1886 1950

1963 1974

Author

Kraus F. Hollinger et al

McAlpine Present case

51 40

74

Age Autopsy Bronchoscopy Laryngoscopy Laryngoscopy

Trachea Trachea Trachea Trachea

Male Male

Female Female

Diagnosis

Site

Sex

Dyspnoea Dyspnoea and cough

No data Wheeze

Symptoms

CLINICAL FINDINGS IN 4 CASES OF AMYLOIDOSIS OF TRACHEA

TABLE

Pneumonia Alive Alive Alive

2 years 2 years

Cause of death

No data 2 years

Duration

o

Q

S. G. Deodhare and G. Dasgupta osis of the trachea. Although rare, this condition should be considered in the differential diagnosis of stenosing lesions and neoplasms of the trachea. This is particularly important, since patients with this lesion may derive significant relief from the symptoms and signs of air-way obstruction by repeated bronchoscopy and removal of the areas of greatest involvement (Domm et al., 1965). Differentiation of solitary amyloidosis from other lesions, especially neoplasms, on clinical grounds alone is not possible and the diagnosis rests mainly on pathologic examination of the specimen. Special staining techniques exist to identify amyloid. Congo red stained sections when viewed under polarised light, exhibit a characteristic green birefringence which is the sine qua non for the identification of amyloid at the level of the optical microscope (Heller et al., 1964). When this method is used with the flurochrome Thioflavine—T staining method, false positives can be excluded. Both Congo red and Thioflavine—T staining reactions were positive for amyloid in our case. The treatment of tracheal amyloidosis is basically removal of the obstructing amyloid substance. Piecemeal removal of projecting masses by bronchoscopic biopsy is the most satisfactory form of management in tracheobronchial amyloidosis (Domm et al., 1965). The operative procedure of the present case is similar in principle to that of McAlpine et al. (1963). In addition to their procedure, we made a window in the region of first and second tracheal rings to have a better view of the subglottic region. We have also done skin grafting of the raw area to promote early healing. However our procedure was rendered difficult, because of prior tracheostomy which was unavoidable. Redeposition of amyloid in the tracheobronchial tree occurs at an unpredictable rate and degree, and recurrences are frequent (Gottlieb and Gold, 1972). Summary A case of primary amyloidosis of the trachea in a 40-year-old female is described. It appeared clinically as a tumour-like lesion in the trachea. The specificity of amyloid deposits was confirmed by a positive reaction to Congo red stained sections viewed under polarised light and Thioflavine—T stained section showing characteristic fluorescence. Excision of the lesion with a split thickness graft was the mode of treatment. Acknowledgements We should like to thank Dr. Leonard Bases, from New York for helping us in the management of the case. Our thanks are also due to Dr. R. K. Kolhatkar, Director, Wanless Hospital and Miraj Medical Centre, for allowing us to publish the case. REFERENCES ANTUNES, M., and VIEIRA DA LUZ, J. M. (1969) Thorax, 24, 307. BURNS, J., PENNOCK, C. A., and STOWARD, P. J. (1967) Journal

Bacteriology, 94, 337. 650

of Pathology and

Clinical records B. M., VASSALLO, C. L., and ADAMS, C. L. (1965) American Journal of Medicine, 38, 151. GOTTLIEB, L. S., and GOLD, W. M. (1972) American Review of Respiratory Disease, 105, 425. HELLER, H., MISSMAHL, H. P., SOHAR, E., and GAFNI, J. (1964) Journal of Pathology and Bacteriology, 88, 15. HOLINGER, P. H., NOVAK, F. G., and JOHNSTON, K. C. (1950) Laryngoscope, 60, DOMM,

1086.

MCALPINE,

J. C,

RADCLIFF,

A., and

FRIEDMANN,

and Otology, 77, 2.

Wanless Hospital, Miraj Medical College, Miraj, Maharashtra, India.

651

I. (1963) Journal of Laryngology

Primary amyloidosis of the trachea.

A case of primary amyloidosis of the trachea in a 40-year-old female is described. It appeared clinically as a tumour-like lesion in the trachea. The ...
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