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| Year : 1983 | Volume
: 29
| Issue : 4 | Page : 255-8 |
Aggressive lymphangioma (report of an unusual case).
Deodhare SG, Siddiqui HM
How to cite this article:
Deodhare S G, Siddiqui H M. Aggressive lymphangioma (report of an unusual case). J Postgrad Med 1983;29:255 |
Lymphangioma is a tumour-like lesion of lymphatics, consisting of endothelium-lined dilated lymphatic channels containing lymph. It is not settled whether the lesion represents a malformation, lymphangiectasis, or benign neoplasm. Four histological types are recognised. 1. Simple lymphangioma. 2. Cystic lymphangioma, 3. Cavernous lymphangioma and 4. Lymphangioma hemangioma. Two related neoplasms are also recognised-lymphangiopericytoma (or lymphangiomyoma) and lymphangiosarcoma. With the exception of the last named, all others behave as benign lesions.
Cystic lymphangioma (cystic hygroma) and cavernous lymphangioma are two most important lesions of lymphatics in children. Cystic hygromas are multilocular cystic lesions which most frequently occur in the neck and are transilluminant. Cavernous lymphangiomas are predominantly solid with a sponge like net-work of small channels and cysts measuring a few millimetres in diameter. The latter are less amenable to complete surgical excision than the former, as they often show extensions into the adjacent muscles and fibrous tissue, and consequently recur following incomplete removal. Both lesions are otherwise benign and do not metastasise.
We are reporting here a lymphangioma in a young child, which showed cystic and cavernous lymphatic channels, with unusual stromal cellularity, early recurrence and lethal behaviour. The benign appearance of this tumour was difficult to correlate with its clinical behaviour and raised the question of a possible malignant transformation.
G. S. M., a 1½ year old Hindu male child, presented with a swelling of the left side of the neck for the past 1½ months, beginning in February 1975. Physical examination revealed good general health without any systemic signs. There was a 7.5 cm x 7.5 cm, lobulated swelling on the left side of neck, with a smooth outer surface, lying deep to the sternomastoid muscle. The swelling was non-pulsatile, fluctuant, and transilluminant. It was excised with the provisional clinical diagnosis of cystic hygroma or branchial cyst.
At operation, the surgeon encountered a fibrous cyst wall at the periphery of the lesion. Prior to excision, a needle aspiration of the lesion yielded thin, brownish fluid. On excision, the cyst was seen to contain dirty, friable material. A portion of the cyst wall adherent to the carotid vessels posteriorly could not be excised and was left behind after excising the maximum possible amount of the lesion.
Post-operatively, the wound healed well without any obvious residual swelling. Examination of the excised material revealed several irregular pieces of soft tissue, with rough, greyish-pink surfaces. On further sectioning, pale homogenous or fibrillar greyish tissue was revealed. Histopathologic examination revealed a lesion which was interpreted as "lymphoma of reticulum cell type, associated with possibly a cystic hygroma". About a month later, a few nodules became palpable on the left side of the neck; these were firm and fixed to the deeper structures. The child was hospitalised and given a course of deep X-ray therapy by single field, delivering 3100 rads to the overlying skin in the course of 4 weeks. Cyclophosphamide was also given in a dose of 25 mg biweekly. A significant reduction in the tumour size was noted. An X-ray film of the chest showed no abnormality. Examination of blood showed severe hypechromic anemia with normal total and differential leukocyte counts throughout the patient's hospital stay. Bone marrow aspirations failed to yield marrow fragments.
Seven weeks later, the patient was hospitalised for the second time. Inspite of continued cyclophosphamide therapy, the swelling on the left side of the neck had reappeared and measured 6 cm in its long axis. It was also fixed to the muscles. There was no dysphagia or dyspnoea. The liver and spleen were not palpable. The hemogram revealed persistent severe anemia, with normal leukocyte and platelet counts. X-ray film of the chest still showed no abnormality. The child was put on a higher dose of cyclophospbamide ( tablet daily) and discharged.
Nearly eight weeks later, the child was hospitalised once again, with an increase in the size of the cervical swelling, which now extended from the angle of the left mandible to the infraclavicular area. It was soft to firm, non-tender, and fluctuant posteriorly. Dysphagia was noticed for 2 weeks prior to admission. Severe anemia persisted. Other blood parameters remained normal. X-ray film of the chest was still unremarkable. Screening of the chest and neck showed soft shadows in the left upper lobe and apex and a soft tissue mass in the left cervical region. The child became progressively weaker and dyspnoeic, and expired in December 1975, eleven months after the onset of symptoms.
Baffled by the behaviour of the tumor in our case, we reviewed the pretreatment histology slides of the excised cervical tumor and observed the following features: A fibrous capsule (or cyst wall) seperated the tumor from the adjacent adipose tissue. A part of the tumor consisted of large, irregularly dilated, lymphatic channels lined by endothelial cells, set in a collagenous, acellular stroma. Lymphocytes were seen in some spaces but there were no red cells. Other areas of the tumor showed fewer dilated lymphatics, and a host of smaller channels lined by prominent endothelial cells. The stroma in these areas was richly cellular and was composed of sheets of medium-sized cells with scanty cytoplasm and single, large, round or oval nuclei. There was little pleomorphism. The nuclear chromatin was clumped in a few blocks and mitoses were inconspicuous both in the endothelial cells and in the stromal cells. Reticulin stain revealed a rich net-work of delicate reticulin fibres in the walls of lymphatics, and in the stroma surrounding small clusters of cells and individual cells. Clear-cut spindle cells or elements of normal or altered lymph nodes were not seen. Tumor necrosis was conspicuous in several areas. [fig 1],[fig 2],[fig 3],[fig 4].
Interpretation of the histopathologic findings posed a problem. A lymphangioma with a combination of cystic and cavernous lymphatics came readily to mind, but the presence of cellular elements in sheets separating the lymphatic channels, was an unusual feature. However, such stromal cellularity is described in lymphangiomas (with or without admixed hemangioma) showing conspicuous endothelial proliferation and aggregates of lymphocytes.[9] In our case, the distribution of reticulin fibres also pointed to a lymphangioma with unusual stromal cellularity. We did not find definite evidence in support of the original diagnosis of a "lymphoma of reticulum cell type". The stromal cells resembled large lymphoid cells but were mature looking, devoid of atypia, and did not appear to invade the capsule and neighbouring tissues. The adherence of the cyst wall to blood vessels and the recurrence of the lesion following surgical excision resembled the well-documented behaviour of cavernous lymphangioma. The tendency of this tumor to penetrate and destroy the surrounding tissues and to recur following surgical excision constitutes the most difficult problem in its management.[1], [6] As for malignant lymphoma, we have not yet encountered a case, and are also not aware of any reported instance, where lymphangioma was associated with a malignant lymphoma in the same lesion. Repeated chest X-ray films did not reveal any evidence of mediastinal lymphoma.
Lyrrmphangiopericytoma (or lymphangiomyoma) and lymphangiosarcoma were also considered in differential diagnosis, chiefly on account of the rapid progress, recurrence, and early fatality. Lymphangiosarcoma is a rare and highly invasive tumor which is most commonly associated with chronic lymphoedema of an extremity e.g. post-mastectomy lymphoedema of the upper extremity[3],[5],[11],[12],[13] with the exception of filarial lymphoedema. Less commonly, it also arises de novo, as well as in irradiated lymphangiomas.[1] It reportedly does not occur in children, and in the head-neck region.[1],[6] Lymphangiopericytoma exhibits a striking female preponderance. It is usually intrathoracic, associated with chylothorax and pulmonary involvement, but is not known to invade or metastasize. Histologically, it shows smooth muscle cells proliferating around and in between the lymphangiomatous channels[2], [4], [8] A more widespread disorder of this type was described by Joliat and associates[7] as lymphangiomyomatosis. In our case, distinct spindle cells with or without cellular atypia were not found and there was no evidence of chylothorax.
Kaposi's sarcoma and proliferating angioendotheliomatosisi[10] were also considered, but appeared unlikely.
The absence of involvement of lymph nodes, liver, spleen and other organs is strongly against the possibility of a lymphoma or a highly malignant angiosarcoma. However, the initial response to radiotherapy and antitumor chemotherapy, and the subsequent recurrence and continued growth of the lesion suggest several possibilities. Was it only a cavernous lymphangioma with its usual tendency to infiltrate locally and to recur following removal? Was there an element of lymphangiopericytoma as well which was not represented in the diagnostic surgical pathology sections, and which subsequently grew rapidly and proved lethal due to local pressure effects? Was there perhaps a malignant transformation, or at least an accelerated phase of growth in the original cavernous lymphangioma (or lymphangiopericytoma)? We have no definite answer to any of these questions, but the case continues to intrigue us.
We are grateful to Dr. D. G. Ohol, M.D., D.P.B., Consultant Pathologist, Dr. A. B. Raval, M.S., Practising Surgeon, and Dr. S. M. Natekar, M.D., Radiologist, Civil Hospital; all from district Sangli (M.S.), for their most valuable help in providing clinical data, diagnostic material and expert advice on various aspects of this case. We also wish to thank the Director, Wanless Hospital and Miraj Medical Center, Miraj (Sangli, M.S.) for his permission to use the Hospital Records of this case.
| 1. | Batsakis, J. G.: Vasoformative tumors. In, "Tumours of the Head and Neck Clinical and Pathological Considerations", 2nd Edition, The Williams and Wilkins Co., Baltimore, 1979, pp. 301-303.  |
| 2. | Cornog, J. L. Jr. and Enterline, H. T.: Lymphangiomyoma: A benign lesion of chyliferous lymphatics synonymous with lymphangio-pericytoma. Cancer, 19: 1909-1930, 1966. |
| 3. | Eby, C. S., Brennan, M. J. and Fine, G.: Lymphangio-sarcoma-a lethal complication of chronic lymphoedema. Report of 2 cases and review of the literature. Arch. Surg., 94: 223-230, 1967. |
| 4. | Enterline, H. T. and Roberts, B.: Lymphangiopericytoma; Case report of a previously undescribed tumor. Cancer, 8: 582-587, 1955. |
| 5. | Finlay-Jones, L. R.: Lymphangiosarcoma of the thigh; A case report. Cancer, 26: 722-725, 1970. |
| 6. | Harkins, G. A. and Sabiston, Ds C. Jr.: Lymphangioma in infancy and childhood. Surgery, 47: 811-822, 1960. |
| 7. | Joliat, G., Stadler, Hr and Kapanci, Y.: Lymphangiomyomatosis-A clinico-anatomical entity. Cancer, 31: 455-461, 1973. |
| 8. | Pachter, M. R. and Lattes, R.: Mesenchymal tumours of the mediastinum. III-Tumours of lymph-vascular origin. Cancer, 16: 108-117, 1963. |
| 9. | Park, W. W. and Corkhill, J. W.: Some miscellaneous lesions. In, "the Histology of Borderline Cancer." Springer-Verlag, Berlin, Heidelberg, New York, 1980, pp. 433-436. |
| 10. | Scott, P. W. B., Silvers, D. N. and Hellwig, F. B.: Proliferating angioendotheliomatosis. Arch. Pathol., 99: 323-326, 1975. |
| 11. | Stewart, F. W. and Treves, N.: Lymphangiosarcoma in postmastectomy lymphoedema. Cancer, 1: 64-81, 1948. |
| 12. | Stout, A. P.: Tumours of the soft tissues. In, "Atlas of Tumour Pathology." Fascicle 5, Section-2, Armed Forces Institute of Pathology, Washington, D.C., 1953, p. 13. |
| 13. | Taswell, H. F., Soule, E. H. and Coventry, M. B.: Lymphangiosarcoma arising in chronic lymphoedematous extremities. Report of 13 cases and review of the literature. J. Bone & Joint Surg., 44-A: 277-294, 1962. |
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