Clinical records Hamartomas of the nose By S. MAHINDRA. R. DALJIT. M. A. SOHAIL and
G. BAZAZ MALIK (New Delhi, India)
THE term hamarloma was introduced by Albrecht (1904) for tumour-like malformations resulting from inborn errors of tissue development, characterized by abnormal admixtures of mature tissues indigenous to the part. Hamartomas may be derived from any of the germinal layers and any type of tissue may predominate. Hamartomas frequently arise from the skin and subcutaneous tissues, kidney, lung, liver, spleen, lips, tongue and G.T.Tract. These tumours, however, arc very rarely encountered in otolaryngological practice. Ishii et al. (1968) reported a case arising from the tongue. Climie el ah (1964) published a single case of lymphoid hamartoma of the larynx. Eichel and Hallberg (1966) reported another case in which the tumour originated in the Eustachian tube and involved the middle ear. Kackcr and Das Gupta (1973) reported the only case of hamartoma arising from the nose which is available in the English literature. The patient presented with a bleeding polypoidal mass in the nose and histological examination revealed an angiomatous hamartoma. Three such cases encountered in this hospital are being reported here because of the rarity of the condition. Cases Case I. A female child age 10 years attended the E.N.T. out-patients on 19.10.68 complaining of bleeding from the right side of the nose for 15 days. The bleeding was profuse and spontaneous. There was no history of nasal discharge, injury, or recurrent colds. On general examination, she was a healthy child, not anaemic. Examination of the nose revealed a pinkish smooth mass, \ in. x A in. in diameter, arising from the right side of the septum which bled on touch. A few small discrete lymph nodes could be palpated in the submandibular region. The routine laboratory investigations were within normal limits. X-ray P.N.S. was normal. The mass was excised on 8.11.68 and bleeding controlled by anterior nasal packing. The histopathology report was of an angiomatous hamartoma (Fig. 1). Case 2. A 26-year-old female was admitted on 3.8.73 with the complaints of nasal obstruction and recurrent bleeding from the nose for two months. She had also noticed a swelling on the left side of the nose for 1£ months. On general examination, she was a healthy young woman. There was deformity of the external nose. Anterior rhinoscopy revealed a pinkish fleshy mass with a smooth surface, filling the entire nasal cavity and reaching the posterior choana, which bled on touch. Routine laboratory investigations and X-ray P.N.S. were normal. The mass was excised. There was profuse bleeding which required nasal packing. The histopathology report was inflammed nasal polyp, an angiomatous hamartoma. 57
FIG. 1 Shows numerous capillaries lined by endothelial cells separated by young connective tissue (>:400).
FIG. 2 Clinical photograph of Case 3.
CLINICAL RECORDS
59
Case 3. A two-month-old baby was brought to the E.N.T. out-patients on 17.8.76 with a small swelling arising from the right anterior nares since birth. It was gradually increasing in size. Nasal obstruction was frequently noticed by the mother. On general examination, she was a healthy baby. There was a smooth, lobular, sessile swelling arising from the medial wall of the right vestibule of the nose involving the anterior nares on the right side (Fig. 2). Its surface was smooth and the skin covering ii appeared normal. There was no ulceration. Routine laboratory investigations were normal. The mass was excised and the histopathology report was lipomatous hamartoma (Fig. 3). It recurred within four months of excision and had to be re-excised. Comments
According to Willis (1953) the term hamartoma should be applied only to lesions for which there is definite evidence of developmental anomaly, either actual malformation with tissue excess already present at birth or an inborn tissue anomaly which manifests itself by excessive growth until the age of puberty. The nomenclature depends on the tissue type which predominates, thus in vascular predominance it is angiomatous, in fatty tissue predominance it is lipomatous, and if lymphoid tissue predominates it is lymphomatous hamartoma. Eichel and Hallberg (1966) have differentiated hamartomas from teratomas and dermoids. The term teratoma implies spontaneous, autonomous, newgrowth derived from pluripotent tissues. It is foreign to the region in which it occurs and is composed of elements of all the three germinal layers. A dermoid tumour has the same histogenesis but differs in that it is usually cystic and, unlike teratoma, originates from only two germinal layers, the ectoderm and the mesoderm. Clinically it is difficult to differentiate between teratomas, dermoids, when these are small, and hamartomas.
FIG. 3 Shows dermal appendages and lobules of adipose tissue separated by connective tissue (xlOO).
60
S. MAHINDRA, R. DALJIT, M. A. SOHAIL AND G. B. MALIK
In the first two cases in the present series the vascular tissue predominated; these were angiomatous hamartomas (Fig. 1). Each of these consisted of thin- and thick-walled vascular channels and spaces. Only if the mass was present at birth or appeared before complete development of the organ, may a provisional diagnosis of hamartoma be kept in mind. Congenital haemangiomatous hamartomas arising from the nose may also present a similar picture. The histopathological features may also be similar in both cases. The third case in the present series, reported as lipomatous hamartoma (Fig. 3) belongs to the group of adipose hamartomas in Willis's classification of hamartomas. In this case, the mass was arising from the skin and superficial tissues of the nasal vestibule. It recurred within four months of complete surgical excision and gradually attained its initial size, requiring wide excision. Summary
Three cases of hamartomas of the nose, very rare conditions in otolaryngology are reported. According to the Willis (1953) classification two of these cases, belonged to the angiomatous, and the third one to the lipomatous, group of hamartomas. Acknowledgement
We are grateful to Dr. A. Dass, M.R.C.O.G., Principal and Medical Superintendent of Lady Hardinge Medical College and Hospital, New Delhi, India, for her kind permission to publish this paper. REFERENCES ALBRECHT, E. (1904) Verhandhmgen der Deutschen pathologischcn Cescllschafl, 7, 153. Cited by R. A. Willis (1958). CLIMIE, A. R. W., WAGGONER, L. G., and KRABBENHOFT, K. I.. (1964) Laryngoscope, 74, 1381.
EICHEL, B. S., and HALLBERG, O. E. (1966) Laryngoscope, 76, 1810.
EVANS, R. VV. (1956) Histological Appearances of Tumours. Edinburgh: Livingstone. FULLER, A. P. (1966) Journal of Laryngology and Otology, 77, 706. ISHII, T., TAKEMORI, S., and SUZUKI, J. I. (1968) Archives of Otolaryngology, 88, 171.
KACKER, S. K., and DAS GUPIA, G. (1973) Journal of Laryngology and Otology, 81, 801. WILLIS, R. A. (1953) Pathology of Tumours, 2nd ed. London: Butterworth. WILLIS, R. A. (1958) Borderland of Embryology and Pathology. London: Butlerworth. Department of Ear, Nose and Throat, Lady Hardinge Medical College and Hospital, New Delhi, India.
G. BAZAZ MALIK (New Delhi, India)
THE term hamarloma was introduced by Albrecht (1904) for tumour-like malformations resulting from inborn errors of tissue development, characterized by abnormal admixtures of mature tissues indigenous to the part. Hamartomas may be derived from any of the germinal layers and any type of tissue may predominate. Hamartomas frequently arise from the skin and subcutaneous tissues, kidney, lung, liver, spleen, lips, tongue and G.T.Tract. These tumours, however, arc very rarely encountered in otolaryngological practice. Ishii et al. (1968) reported a case arising from the tongue. Climie el ah (1964) published a single case of lymphoid hamartoma of the larynx. Eichel and Hallberg (1966) reported another case in which the tumour originated in the Eustachian tube and involved the middle ear. Kackcr and Das Gupta (1973) reported the only case of hamartoma arising from the nose which is available in the English literature. The patient presented with a bleeding polypoidal mass in the nose and histological examination revealed an angiomatous hamartoma. Three such cases encountered in this hospital are being reported here because of the rarity of the condition. Cases Case I. A female child age 10 years attended the E.N.T. out-patients on 19.10.68 complaining of bleeding from the right side of the nose for 15 days. The bleeding was profuse and spontaneous. There was no history of nasal discharge, injury, or recurrent colds. On general examination, she was a healthy child, not anaemic. Examination of the nose revealed a pinkish smooth mass, \ in. x A in. in diameter, arising from the right side of the septum which bled on touch. A few small discrete lymph nodes could be palpated in the submandibular region. The routine laboratory investigations were within normal limits. X-ray P.N.S. was normal. The mass was excised on 8.11.68 and bleeding controlled by anterior nasal packing. The histopathology report was of an angiomatous hamartoma (Fig. 1). Case 2. A 26-year-old female was admitted on 3.8.73 with the complaints of nasal obstruction and recurrent bleeding from the nose for two months. She had also noticed a swelling on the left side of the nose for 1£ months. On general examination, she was a healthy young woman. There was deformity of the external nose. Anterior rhinoscopy revealed a pinkish fleshy mass with a smooth surface, filling the entire nasal cavity and reaching the posterior choana, which bled on touch. Routine laboratory investigations and X-ray P.N.S. were normal. The mass was excised. There was profuse bleeding which required nasal packing. The histopathology report was inflammed nasal polyp, an angiomatous hamartoma. 57
FIG. 1 Shows numerous capillaries lined by endothelial cells separated by young connective tissue (>:400).
FIG. 2 Clinical photograph of Case 3.
CLINICAL RECORDS
59
Case 3. A two-month-old baby was brought to the E.N.T. out-patients on 17.8.76 with a small swelling arising from the right anterior nares since birth. It was gradually increasing in size. Nasal obstruction was frequently noticed by the mother. On general examination, she was a healthy baby. There was a smooth, lobular, sessile swelling arising from the medial wall of the right vestibule of the nose involving the anterior nares on the right side (Fig. 2). Its surface was smooth and the skin covering ii appeared normal. There was no ulceration. Routine laboratory investigations were normal. The mass was excised and the histopathology report was lipomatous hamartoma (Fig. 3). It recurred within four months of excision and had to be re-excised. Comments
According to Willis (1953) the term hamartoma should be applied only to lesions for which there is definite evidence of developmental anomaly, either actual malformation with tissue excess already present at birth or an inborn tissue anomaly which manifests itself by excessive growth until the age of puberty. The nomenclature depends on the tissue type which predominates, thus in vascular predominance it is angiomatous, in fatty tissue predominance it is lipomatous, and if lymphoid tissue predominates it is lymphomatous hamartoma. Eichel and Hallberg (1966) have differentiated hamartomas from teratomas and dermoids. The term teratoma implies spontaneous, autonomous, newgrowth derived from pluripotent tissues. It is foreign to the region in which it occurs and is composed of elements of all the three germinal layers. A dermoid tumour has the same histogenesis but differs in that it is usually cystic and, unlike teratoma, originates from only two germinal layers, the ectoderm and the mesoderm. Clinically it is difficult to differentiate between teratomas, dermoids, when these are small, and hamartomas.
FIG. 3 Shows dermal appendages and lobules of adipose tissue separated by connective tissue (xlOO).
60
S. MAHINDRA, R. DALJIT, M. A. SOHAIL AND G. B. MALIK
In the first two cases in the present series the vascular tissue predominated; these were angiomatous hamartomas (Fig. 1). Each of these consisted of thin- and thick-walled vascular channels and spaces. Only if the mass was present at birth or appeared before complete development of the organ, may a provisional diagnosis of hamartoma be kept in mind. Congenital haemangiomatous hamartomas arising from the nose may also present a similar picture. The histopathological features may also be similar in both cases. The third case in the present series, reported as lipomatous hamartoma (Fig. 3) belongs to the group of adipose hamartomas in Willis's classification of hamartomas. In this case, the mass was arising from the skin and superficial tissues of the nasal vestibule. It recurred within four months of complete surgical excision and gradually attained its initial size, requiring wide excision. Summary
Three cases of hamartomas of the nose, very rare conditions in otolaryngology are reported. According to the Willis (1953) classification two of these cases, belonged to the angiomatous, and the third one to the lipomatous, group of hamartomas. Acknowledgement
We are grateful to Dr. A. Dass, M.R.C.O.G., Principal and Medical Superintendent of Lady Hardinge Medical College and Hospital, New Delhi, India, for her kind permission to publish this paper. REFERENCES ALBRECHT, E. (1904) Verhandhmgen der Deutschen pathologischcn Cescllschafl, 7, 153. Cited by R. A. Willis (1958). CLIMIE, A. R. W., WAGGONER, L. G., and KRABBENHOFT, K. I.. (1964) Laryngoscope, 74, 1381.
EICHEL, B. S., and HALLBERG, O. E. (1966) Laryngoscope, 76, 1810.
EVANS, R. VV. (1956) Histological Appearances of Tumours. Edinburgh: Livingstone. FULLER, A. P. (1966) Journal of Laryngology and Otology, 77, 706. ISHII, T., TAKEMORI, S., and SUZUKI, J. I. (1968) Archives of Otolaryngology, 88, 171.
KACKER, S. K., and DAS GUPIA, G. (1973) Journal of Laryngology and Otology, 81, 801. WILLIS, R. A. (1953) Pathology of Tumours, 2nd ed. London: Butterworth. WILLIS, R. A. (1958) Borderland of Embryology and Pathology. London: Butlerworth. Department of Ear, Nose and Throat, Lady Hardinge Medical College and Hospital, New Delhi, India.
