Diagnostic Radiology
Lymphographic Demonstration of a Retroperitoneal Lymphangioma 1 Ronald A. Castelllno, M.D. and Solon Finkelstein, M.D. Lymphangiomas are rare, benign tumors which may become opacified during lymphography and may be confused with malignant lymph node disease. A case is reported in which lymphography showed focal collections of contrast material retained for several days in dilated lymphatic spaces which initially simulated metastatic disease. INDEX TERMS:
Lymphangioma. Lymphatic system, neoplasms
Radiology 115:355-356, May 1975
in whom a small retroperitoneal lymphangioma was found as an incidental finding at lymphography is the subject of this report. The appearance of the lymphogram caused by the lymphangioma was such that an erroneous diagnosis of metastasis to the periaortic lymph nodes was made. «PATIENT
M
CASE REPORT This 37-year-old white woman had a 2.5-cm right tracheobronchial mass on a routine chest radiograph. Pathologic examination of the resected yellowish, lobulated tumor showed an undifferentiated carcinoma, perhaps arising in the thymus. An extensive search for a possible primary site was unrewarding. Subsequent evaluation included bilateral lower extremity lymphography. The left upper periaortic lymphatic channels were displaced laterally, as were several enlarged but otherwise normal appearing lymph nodes. Twenty-four hours following lymphography, there was irregular opacification over a 2 X 5-cm area in the left periaortic region which suggested lymphographic contrast agent within enlarged, markedly displaced lymph nodes (Fig. 1). This appearance was compatible with metastasis, but radiographs three days later showed interval disappearance of most of this contrast material, suggesting, in retrospect, that it was within abnormal lymphatic channels or spaces rather than in lymph nodes. Laparotomy 12 days later revealed a multiloculated cystic structure overlying the aorta and spine, which caused anterior and lateral displacement of the periaortic lymph nodes. On biopsy this proved to be a lymphangioma containing numerous dilated spaces lined by apparent endothelial cells and fibromuscular tissue, in close relation to afferent lymphatics of histologically unremarkable lymph nodes. At 9 or 10 years of age the patient had had an undue number of fractures. Radiographs reportedly showed multiple lytic lesions involving skull, ribs, forearms and pelvis. These were diagnosed clinically as "xanthomatosis." No biopsy was performed. She was treated with radiotherapy and subsequent radiographs at age 13 reportedly showed most of these lesions to be healed. As a young child, she had a •'thyroid abnormality," a condition her father also suffered. Since age 13, she has had idiopathic edema, primarily in the legs.
Current physical examinations have been normal except for 1+ pretibial edema. She has remained free of disease for three years following localized radiotherapy to the mediastinum. A brother died at age 4 from what was considered to be lymphosarcoma, with an illness characterized by ascites and pleural effusions containing large numbers of lymphocytes; a lymph node biopsy showed large lymphocytes, many in mitoses, with reticulum fibers on silver stain.
DISCUSSION Lymphangiomas (3, 5, 6, 8) are rare, benign tumors of the lymphatic system, composed of multiple lymphatic spaces often lined by endothelium and containing fibromuscular septa. They are classified as being simple, cavernous or cystic. They may be single or multiple, unilocular or multilocular, and may contain serous or chylous fluid. Although the etiology of lymphangioma is not known, most investigators favor a congenital origin due to aberrant rests of lymphoid tissue which do not develop a vascular communication. This tumor may attain a size sufficient to cause symptoms by displacement or compression of adjacent structures. These tumors often are noted as incidental findings at surgery or autopsy, or are discovered as incidental findings on radiographic studies. Lymphangiomas are more commonly seen in the neck (where they are called cystic hygroma if of the cystic type), axillae, and extremities, as well as in the mediastinum, retroperitoneum and mesentery (where they are often called mesenteric cysts). Lymphangiectasis, characterized by masses of lymphatic cysts, shares certain similarities with the spectrum of lymphangiomas (1). Lymphangiomas also occur in bone and may produce bone absorption. Whether the reported lytic bone lesions in this patient were caused by lymphangiomas is speculative. Lymphography can directly visualize only those lymphangiomas which communicate with the lymphatic system. Lower extremity soft-tissue lymphangiomas have shown numerous, variable-sized intercommunicating lymphatic channels and spaces in the area of the lymphangiomatous growth (4, 5). Lymphangiomas involving bone may be opacified during lymphography as well (9). A cystic lymphangioma of the mesentery (mesenteric cyst), which was shown to communicate with the thoracic duct by direct injection, demonstrated dilatation of numerous interconnecting lymphatic channels with passage of contrast material into the mesenteric cystic spaces on lower extremity lymphography (7). Direct injection of a penile and scrotal lymphangioma revealed a dense intercommunicating subepidermal lymphatic plexus (2). The localized retroperitoneal lymphangioma in our patient demonstrated irregular opacification of multiple dilated lymphatic spaces. The interval disappearance of much of this contrast material over several days, as seen on follow-up radiographs, should have suggested that the mass was primarily composed of numerous dilated lymphatic spaces, suggesting the diagnosis of a lymphangioma. REFERENCES 1. Gill WM Jr, Alfidi RJ: Roentgenographic manifestations of lymphangiectasis: report of a case. Am J Roentgenol 109: 185-189, May 1970
1 From the Divisions of Diagnostic Radiology, Stanford University School of Medicine, and the Palo Alto Medical Clinic, Palo Alto, Calif. Accepted for publication in November 1974. dk
355
356
RONALD
A. CASTELLINO AND SOLON FINKELSTEIN
May 1975
Fig. 1. Left posterior oblique (left) and anteroposterior (middle) radiographs 24 hours following bilateral lower extremity lymphography. Note the bizarre collections of contrast material in the upper left periaortic region (arrows) which represent globules of contrast medium in dilated lymphatic spaces. Anteroposterior (right) radiograph 3 days later shows interval disappearance of most of the globules of contrast medium, although scattered collections remain (arrows).
2. Greiner R, Schmid GH: Lymphography of the skin of the scrotum and of the penis in a case of lymphangioma circumscriptum cysticum. Lymphology 4: 140-144, Dec 1971 3. Henzel JH, Pories WJ, Burget DE, et al: Intra-abdominallymphangiomata. Arch Surg 93:304-308, Aug 1966 4. Kittredge RD, Finby N: The many facets of lymphangioma. Am J RoentgenoI95:56-66, Sep 1965 5. Kuisk H: Technique of Lymphography and Principles of Interpretation. St. Louis, Warren H. Green, 1971, pp 204-206 6. Larson DL, Myhre BA, Schmidt ER, et al: Lymphangioma in unusual sites: spleen, mesentery, retroperitoneum, mediastinum and the greater omentum. Wis Med J 60:279-287, May 1961
7. Leonidas JC, Kopel FB, Danese CA: Mesenteric cyst associated with protein loss in the gastrointestinal tract: study with lymphangiography. Am J Roentgenol 112: 150-154, May 1971 8. Rauch RF: Retroperitoneal lymphangioma. Arch Surg 78: 45-50, Jan 1959 9. Winterberger AR: Radiographic diagnosis of Iymphangiomatosis of bone. Radiology 102:321-324, Feb 1972
Department of Radiology Stanford University School of Medicine Stanford, Calif. 94305
Lymphographic Demonstration of a Retroperitoneal Lymphangioma 1 Ronald A. Castelllno, M.D. and Solon Finkelstein, M.D. Lymphangiomas are rare, benign tumors which may become opacified during lymphography and may be confused with malignant lymph node disease. A case is reported in which lymphography showed focal collections of contrast material retained for several days in dilated lymphatic spaces which initially simulated metastatic disease. INDEX TERMS:
Lymphangioma. Lymphatic system, neoplasms
Radiology 115:355-356, May 1975
in whom a small retroperitoneal lymphangioma was found as an incidental finding at lymphography is the subject of this report. The appearance of the lymphogram caused by the lymphangioma was such that an erroneous diagnosis of metastasis to the periaortic lymph nodes was made. «PATIENT
M
CASE REPORT This 37-year-old white woman had a 2.5-cm right tracheobronchial mass on a routine chest radiograph. Pathologic examination of the resected yellowish, lobulated tumor showed an undifferentiated carcinoma, perhaps arising in the thymus. An extensive search for a possible primary site was unrewarding. Subsequent evaluation included bilateral lower extremity lymphography. The left upper periaortic lymphatic channels were displaced laterally, as were several enlarged but otherwise normal appearing lymph nodes. Twenty-four hours following lymphography, there was irregular opacification over a 2 X 5-cm area in the left periaortic region which suggested lymphographic contrast agent within enlarged, markedly displaced lymph nodes (Fig. 1). This appearance was compatible with metastasis, but radiographs three days later showed interval disappearance of most of this contrast material, suggesting, in retrospect, that it was within abnormal lymphatic channels or spaces rather than in lymph nodes. Laparotomy 12 days later revealed a multiloculated cystic structure overlying the aorta and spine, which caused anterior and lateral displacement of the periaortic lymph nodes. On biopsy this proved to be a lymphangioma containing numerous dilated spaces lined by apparent endothelial cells and fibromuscular tissue, in close relation to afferent lymphatics of histologically unremarkable lymph nodes. At 9 or 10 years of age the patient had had an undue number of fractures. Radiographs reportedly showed multiple lytic lesions involving skull, ribs, forearms and pelvis. These were diagnosed clinically as "xanthomatosis." No biopsy was performed. She was treated with radiotherapy and subsequent radiographs at age 13 reportedly showed most of these lesions to be healed. As a young child, she had a •'thyroid abnormality," a condition her father also suffered. Since age 13, she has had idiopathic edema, primarily in the legs.
Current physical examinations have been normal except for 1+ pretibial edema. She has remained free of disease for three years following localized radiotherapy to the mediastinum. A brother died at age 4 from what was considered to be lymphosarcoma, with an illness characterized by ascites and pleural effusions containing large numbers of lymphocytes; a lymph node biopsy showed large lymphocytes, many in mitoses, with reticulum fibers on silver stain.
DISCUSSION Lymphangiomas (3, 5, 6, 8) are rare, benign tumors of the lymphatic system, composed of multiple lymphatic spaces often lined by endothelium and containing fibromuscular septa. They are classified as being simple, cavernous or cystic. They may be single or multiple, unilocular or multilocular, and may contain serous or chylous fluid. Although the etiology of lymphangioma is not known, most investigators favor a congenital origin due to aberrant rests of lymphoid tissue which do not develop a vascular communication. This tumor may attain a size sufficient to cause symptoms by displacement or compression of adjacent structures. These tumors often are noted as incidental findings at surgery or autopsy, or are discovered as incidental findings on radiographic studies. Lymphangiomas are more commonly seen in the neck (where they are called cystic hygroma if of the cystic type), axillae, and extremities, as well as in the mediastinum, retroperitoneum and mesentery (where they are often called mesenteric cysts). Lymphangiectasis, characterized by masses of lymphatic cysts, shares certain similarities with the spectrum of lymphangiomas (1). Lymphangiomas also occur in bone and may produce bone absorption. Whether the reported lytic bone lesions in this patient were caused by lymphangiomas is speculative. Lymphography can directly visualize only those lymphangiomas which communicate with the lymphatic system. Lower extremity soft-tissue lymphangiomas have shown numerous, variable-sized intercommunicating lymphatic channels and spaces in the area of the lymphangiomatous growth (4, 5). Lymphangiomas involving bone may be opacified during lymphography as well (9). A cystic lymphangioma of the mesentery (mesenteric cyst), which was shown to communicate with the thoracic duct by direct injection, demonstrated dilatation of numerous interconnecting lymphatic channels with passage of contrast material into the mesenteric cystic spaces on lower extremity lymphography (7). Direct injection of a penile and scrotal lymphangioma revealed a dense intercommunicating subepidermal lymphatic plexus (2). The localized retroperitoneal lymphangioma in our patient demonstrated irregular opacification of multiple dilated lymphatic spaces. The interval disappearance of much of this contrast material over several days, as seen on follow-up radiographs, should have suggested that the mass was primarily composed of numerous dilated lymphatic spaces, suggesting the diagnosis of a lymphangioma. REFERENCES 1. Gill WM Jr, Alfidi RJ: Roentgenographic manifestations of lymphangiectasis: report of a case. Am J Roentgenol 109: 185-189, May 1970
1 From the Divisions of Diagnostic Radiology, Stanford University School of Medicine, and the Palo Alto Medical Clinic, Palo Alto, Calif. Accepted for publication in November 1974. dk
355
356
RONALD
A. CASTELLINO AND SOLON FINKELSTEIN
May 1975
Fig. 1. Left posterior oblique (left) and anteroposterior (middle) radiographs 24 hours following bilateral lower extremity lymphography. Note the bizarre collections of contrast material in the upper left periaortic region (arrows) which represent globules of contrast medium in dilated lymphatic spaces. Anteroposterior (right) radiograph 3 days later shows interval disappearance of most of the globules of contrast medium, although scattered collections remain (arrows).
2. Greiner R, Schmid GH: Lymphography of the skin of the scrotum and of the penis in a case of lymphangioma circumscriptum cysticum. Lymphology 4: 140-144, Dec 1971 3. Henzel JH, Pories WJ, Burget DE, et al: Intra-abdominallymphangiomata. Arch Surg 93:304-308, Aug 1966 4. Kittredge RD, Finby N: The many facets of lymphangioma. Am J RoentgenoI95:56-66, Sep 1965 5. Kuisk H: Technique of Lymphography and Principles of Interpretation. St. Louis, Warren H. Green, 1971, pp 204-206 6. Larson DL, Myhre BA, Schmidt ER, et al: Lymphangioma in unusual sites: spleen, mesentery, retroperitoneum, mediastinum and the greater omentum. Wis Med J 60:279-287, May 1961
7. Leonidas JC, Kopel FB, Danese CA: Mesenteric cyst associated with protein loss in the gastrointestinal tract: study with lymphangiography. Am J Roentgenol 112: 150-154, May 1971 8. Rauch RF: Retroperitoneal lymphangioma. Arch Surg 78: 45-50, Jan 1959 9. Winterberger AR: Radiographic diagnosis of Iymphangiomatosis of bone. Radiology 102:321-324, Feb 1972
Department of Radiology Stanford University School of Medicine Stanford, Calif. 94305
![[Retroperitoneal cystic lymphangioma].](/assets/img/hold.png)
