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Esophageal lymphangiomatosis: A case report
Esophageal lymphangiomatosis
B-I Lee, B-W Kim, K-M Kim, et al.
Esophageal lymphangiomatosis: a case report Bo-In Lee, MD, Byung-Wook Kim, MD, Kyoung-Mee Kim, MD, Ho-Jin Song, MD, Se-Hyun Cho, MD, Hwang Choi, MD, Myung-Gyu Choi, MD, Kyu-Yong Choi, MD, In-Sik Chung, MD, Sang-Bok Cha, MD, Hee-Sik Sun, MD, DooHo Park, MD
Lymphangioma of the GI tract formerly was considered rare, the most frequent sites seemingly being the colon and rectum. It is now known to occur more commonly than previously recognized; more than 100 cases have been reported from Japan alone.1 The esophagus is the least frequent site2 and Current affiliations: Division of Gastroenterology, Departments of Internal Medicine and Clinical Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea. Reprint requests: Byung-Wook Kim, MD, Division of Gastroenterology, Department of Internal Medicine, Our Lady of Mercy Hospital, The Catholic University of Korea, No. 665, Pupyong-dong, Pupyong-gu, Incheon, 403-720, Korea. Copyright © 2002 by the American Society for Gastrointestinal Endoscopy 0016-5107/2002/$35.00 + 0 37/54/128134 doi:10.1067/mge.2002.128134 VOLUME 56, NO. 4, 2002
Figure 1. Initial endoscopic view showing multiple white-yellowish cystic lesions in mid and distal esophagus.
in all cases of esophageal lymphangioma reported to date the lesion was solitary.3 A case of multiple esophageal lymphangioma, so-called esophageal lymphangiomatosis, that was successfully treated by repeated endoscopic unroofing is reported here.4 GASTROINTESTINAL ENDOSCOPY
589
B-I Lee, B-W Kim, K-M Kim, et al.
Esophageal lymphangiomatosis
Figure 4. Endoscopic view immediately after unroofing procedure showing that luminal half of lesion has been removed. Figure 2. Endosonographic image (12 MHz) showing multiple anechoic cyst-like structures with intervening septae predominantly in submucosal layer. Involvement of muscularis propria was suspected but not demonstrated (open arrow).
Figure 5. Endoscopic view 1 month after 3 unroofing sessions; the cystic lesions have disappeared almost completely.
Figure 3. Photomicrograph of biopsy specimen showing multiple dilated lymphatic vessels of varying sizes with lymphoid accumulation (solid arrows) beneath squamous epithelium (open arrow). Arrowheads indicate smooth muscle fibers of muscularis propria (H&E, orig. mag. 100). CASE REPORT A 37-year-old man presented with a 10-day history of dysphagia. Personal medical and family histories were unremarkable. No nausea, vomiting, heartburn, or weight loss was apparent, and standard hematologic and biochemical laboratory tests were within normal limits. EGD revealed 3 large lobulated, translucent, whitishyellow masses with lustrous surface in the mid and distal esophagus (32 to 42 cm from the incisors; Fig. 1). At EUS there were multiple anechoic cyst-like structures with intervening septae, predominantly in the submucosal 590
GASTROINTESTINAL ENDOSCOPY
layer (Fig. 2). Although it was suspected, involvement of the muscularis propria was not demonstrated at EUS. Chest CT with contrast enhancement demonstrated that the cysts were limited to the esophagus. Needle aspiration was attempted at endoscopy but no fluid was obtained. However, adequate specimens were obtained by standard polypectomy, snaring the base of the tumor. The histopathologic findings were consistent with a diagnosis of lymphangioma (Fig. 3). Treatment was initiated with a subcutaneous injection of interferon- 2a (5 million units, 3 times per week), which resulted in a slight improvement in the dysphagia. However, this therapy was stopped after 5 months because of side effects, including myalgia, dry eyes, and loss of hair. EGD after interferon therapy revealed that the lymphangiomas had decreased slightly in size and that their surfaces had become less tense. Nevertheless, the dysphagia persisted. Therefore, a decision was made to “unroof” the lymphangiomas. The tumor VOLUME 56, NO. 4, 2002
Esophageal stricture and dysplasia in squamous cell papillomatosis
was snared about midway between the base and apex to thereby resect the luminal half. A small, oval-shaped electrosurgical snare was used (Olympus SD-12L, Tokyo, Japan) with an electrosurgical unit (Olympus VES-10) set for blend current and an output of 30 W. No complications were encountered because of the unroofing procedure (Fig. 4). Because the middle portion of the mass was not completely removed at the first endoscopic treatment session, because of the presence of multiple septations, 2 more unroofing procedures were performed at 1 month intervals. EGD 1 month after the last procedure showed nearly complete removal of the lesions (Fig. 5). Ranitidine (150 mg twice daily) together with a liquid antacid were administered during the intervals between the unroofing procedures. Over a follow-up period of 10 months the patient has remained symptom free and able to swallow normally.
DISCUSSION Lymphangiomas are benign cystic tumors that contain chylous or serous fluid within an irregularly dilated lymphatic channel. They commonly develop in the head and neck region; only 1% are found in the GI tract. Of these that arise in the gut, the majority are found in the colon followed in order of frequency by stomach, duodenum, small intestine, and esophagus.5 Ten cases of esophageal lymphangioma have been reported,3 the lesions in all cases being solitary. Although a case of multiple lymphangioma of the colon has been reported,1 esophageal lymphangiomatosis, to our knowledge, has not been described in an English language publication. It is probably unnecessary to remove GI lymphangiomas in patients who are asymptomatic because there have been no reports of malignant degeneration. However, in the present case the patient complained of dysphagia and the lesion was large enough to induce this symptom. Although a decision was made to remove the lesion, the length and depth of tissue involvement were excessive, and for this reason an initial attempt at medical therapy was made by using interferon- 2a. It is well-established that
VOLUME 56, NO. 4, 2002
R Narayani, G Young
interferon- has antiproliferative and antiangiogenic properties. Reinhardt et al.6 reported 2 cases of childhood systemic lymphangiomatosis that were treated successfully with interferon- . Although the size and volume of the lymphangiomas decreased slightly, the effect of interferon- was limited in the present case. The plausible causes of this inadequate response are insufficient duration of treatment and the older age of the patient because the pathophysiology of lymphangiomas in children may differ from that in adults. Lymphangiomas can be eliminated by removing the luminal half, a process termed unroofing by Mimura et al.4 Unroofing is a safe method for removal of GI lymphangiomas. In the present case, it proved to be not only safe but a highly effective method of treating esophageal lymphangiomatosis. ACKNOWLEDGEMENT We appreciate the help of LG Chemicals (Seoul, Korea) for providing interferon- 2a (Intermax alpha) without charge for treatment of our patient. REFERENCES 1. Watanabe T, Kato K, Sugitani M, Hasunuma O, Sawada T, Hoshino N, et al. A case of multiple lymphangiomas of the colon suggesting colonic lymphangiomatosis. Gastrointest Endosc 2000;52:781-4. 2. Gangl A, Polterauer P, Krepler R, Kumpan W. A further case of submucosal lymphangioma of the duodenum diagnosed during endoscopy. Endoscopy 1980;12:188-90. 3. Suwa T, Ozawa S, Ando N, Shinozaki H, Tsujitsuka K, Mild H, et al. Case report: lymphangioma of the oesophagus endoscopically resected. J Gastroenterol Hepatol 1996;11:786-8. 4. Mimura T, Kuramoto S, Hashimoto M, Yamasaki K, Kobayashi K, Kobayashi M, et al. Unroofing for lymphangioma of the large intestine: a new approach to endoscopic treatment. Gastrointest Endosc 1997;46:259-63. 5. Hizawa K, Aoyagi K, Kurahara K, Suekane H, Kuwano Y, Nakamura S, et al. Gastrointestinal lymphangioma: endosonographic demonstration and endoscopic removal. Gastrointest Endosc 1996;43:620-4. 6. Reinhardt MA, Nelson SC, Sencer SF, Bostrom BC, Kurachek SC, Nesbit ME. Treatment of childhood lymphangiomas with interferon-alpha. J Pediatr Hematol Oncol 1997;19:232-6.
GASTROINTESTINAL ENDOSCOPY
591
B-I Lee, B-W Kim, K-M Kim, et al.
Esophageal lymphangiomatosis: a case report Bo-In Lee, MD, Byung-Wook Kim, MD, Kyoung-Mee Kim, MD, Ho-Jin Song, MD, Se-Hyun Cho, MD, Hwang Choi, MD, Myung-Gyu Choi, MD, Kyu-Yong Choi, MD, In-Sik Chung, MD, Sang-Bok Cha, MD, Hee-Sik Sun, MD, DooHo Park, MD
Lymphangioma of the GI tract formerly was considered rare, the most frequent sites seemingly being the colon and rectum. It is now known to occur more commonly than previously recognized; more than 100 cases have been reported from Japan alone.1 The esophagus is the least frequent site2 and Current affiliations: Division of Gastroenterology, Departments of Internal Medicine and Clinical Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea. Reprint requests: Byung-Wook Kim, MD, Division of Gastroenterology, Department of Internal Medicine, Our Lady of Mercy Hospital, The Catholic University of Korea, No. 665, Pupyong-dong, Pupyong-gu, Incheon, 403-720, Korea. Copyright © 2002 by the American Society for Gastrointestinal Endoscopy 0016-5107/2002/$35.00 + 0 37/54/128134 doi:10.1067/mge.2002.128134 VOLUME 56, NO. 4, 2002
Figure 1. Initial endoscopic view showing multiple white-yellowish cystic lesions in mid and distal esophagus.
in all cases of esophageal lymphangioma reported to date the lesion was solitary.3 A case of multiple esophageal lymphangioma, so-called esophageal lymphangiomatosis, that was successfully treated by repeated endoscopic unroofing is reported here.4 GASTROINTESTINAL ENDOSCOPY
589
B-I Lee, B-W Kim, K-M Kim, et al.
Esophageal lymphangiomatosis
Figure 4. Endoscopic view immediately after unroofing procedure showing that luminal half of lesion has been removed. Figure 2. Endosonographic image (12 MHz) showing multiple anechoic cyst-like structures with intervening septae predominantly in submucosal layer. Involvement of muscularis propria was suspected but not demonstrated (open arrow).
Figure 5. Endoscopic view 1 month after 3 unroofing sessions; the cystic lesions have disappeared almost completely.
Figure 3. Photomicrograph of biopsy specimen showing multiple dilated lymphatic vessels of varying sizes with lymphoid accumulation (solid arrows) beneath squamous epithelium (open arrow). Arrowheads indicate smooth muscle fibers of muscularis propria (H&E, orig. mag. 100). CASE REPORT A 37-year-old man presented with a 10-day history of dysphagia. Personal medical and family histories were unremarkable. No nausea, vomiting, heartburn, or weight loss was apparent, and standard hematologic and biochemical laboratory tests were within normal limits. EGD revealed 3 large lobulated, translucent, whitishyellow masses with lustrous surface in the mid and distal esophagus (32 to 42 cm from the incisors; Fig. 1). At EUS there were multiple anechoic cyst-like structures with intervening septae, predominantly in the submucosal 590
GASTROINTESTINAL ENDOSCOPY
layer (Fig. 2). Although it was suspected, involvement of the muscularis propria was not demonstrated at EUS. Chest CT with contrast enhancement demonstrated that the cysts were limited to the esophagus. Needle aspiration was attempted at endoscopy but no fluid was obtained. However, adequate specimens were obtained by standard polypectomy, snaring the base of the tumor. The histopathologic findings were consistent with a diagnosis of lymphangioma (Fig. 3). Treatment was initiated with a subcutaneous injection of interferon- 2a (5 million units, 3 times per week), which resulted in a slight improvement in the dysphagia. However, this therapy was stopped after 5 months because of side effects, including myalgia, dry eyes, and loss of hair. EGD after interferon therapy revealed that the lymphangiomas had decreased slightly in size and that their surfaces had become less tense. Nevertheless, the dysphagia persisted. Therefore, a decision was made to “unroof” the lymphangiomas. The tumor VOLUME 56, NO. 4, 2002
Esophageal stricture and dysplasia in squamous cell papillomatosis
was snared about midway between the base and apex to thereby resect the luminal half. A small, oval-shaped electrosurgical snare was used (Olympus SD-12L, Tokyo, Japan) with an electrosurgical unit (Olympus VES-10) set for blend current and an output of 30 W. No complications were encountered because of the unroofing procedure (Fig. 4). Because the middle portion of the mass was not completely removed at the first endoscopic treatment session, because of the presence of multiple septations, 2 more unroofing procedures were performed at 1 month intervals. EGD 1 month after the last procedure showed nearly complete removal of the lesions (Fig. 5). Ranitidine (150 mg twice daily) together with a liquid antacid were administered during the intervals between the unroofing procedures. Over a follow-up period of 10 months the patient has remained symptom free and able to swallow normally.
DISCUSSION Lymphangiomas are benign cystic tumors that contain chylous or serous fluid within an irregularly dilated lymphatic channel. They commonly develop in the head and neck region; only 1% are found in the GI tract. Of these that arise in the gut, the majority are found in the colon followed in order of frequency by stomach, duodenum, small intestine, and esophagus.5 Ten cases of esophageal lymphangioma have been reported,3 the lesions in all cases being solitary. Although a case of multiple lymphangioma of the colon has been reported,1 esophageal lymphangiomatosis, to our knowledge, has not been described in an English language publication. It is probably unnecessary to remove GI lymphangiomas in patients who are asymptomatic because there have been no reports of malignant degeneration. However, in the present case the patient complained of dysphagia and the lesion was large enough to induce this symptom. Although a decision was made to remove the lesion, the length and depth of tissue involvement were excessive, and for this reason an initial attempt at medical therapy was made by using interferon- 2a. It is well-established that
VOLUME 56, NO. 4, 2002
R Narayani, G Young
interferon- has antiproliferative and antiangiogenic properties. Reinhardt et al.6 reported 2 cases of childhood systemic lymphangiomatosis that were treated successfully with interferon- . Although the size and volume of the lymphangiomas decreased slightly, the effect of interferon- was limited in the present case. The plausible causes of this inadequate response are insufficient duration of treatment and the older age of the patient because the pathophysiology of lymphangiomas in children may differ from that in adults. Lymphangiomas can be eliminated by removing the luminal half, a process termed unroofing by Mimura et al.4 Unroofing is a safe method for removal of GI lymphangiomas. In the present case, it proved to be not only safe but a highly effective method of treating esophageal lymphangiomatosis. ACKNOWLEDGEMENT We appreciate the help of LG Chemicals (Seoul, Korea) for providing interferon- 2a (Intermax alpha) without charge for treatment of our patient. REFERENCES 1. Watanabe T, Kato K, Sugitani M, Hasunuma O, Sawada T, Hoshino N, et al. A case of multiple lymphangiomas of the colon suggesting colonic lymphangiomatosis. Gastrointest Endosc 2000;52:781-4. 2. Gangl A, Polterauer P, Krepler R, Kumpan W. A further case of submucosal lymphangioma of the duodenum diagnosed during endoscopy. Endoscopy 1980;12:188-90. 3. Suwa T, Ozawa S, Ando N, Shinozaki H, Tsujitsuka K, Mild H, et al. Case report: lymphangioma of the oesophagus endoscopically resected. J Gastroenterol Hepatol 1996;11:786-8. 4. Mimura T, Kuramoto S, Hashimoto M, Yamasaki K, Kobayashi K, Kobayashi M, et al. Unroofing for lymphangioma of the large intestine: a new approach to endoscopic treatment. Gastrointest Endosc 1997;46:259-63. 5. Hizawa K, Aoyagi K, Kurahara K, Suekane H, Kuwano Y, Nakamura S, et al. Gastrointestinal lymphangioma: endosonographic demonstration and endoscopic removal. Gastrointest Endosc 1996;43:620-4. 6. Reinhardt MA, Nelson SC, Sencer SF, Bostrom BC, Kurachek SC, Nesbit ME. Treatment of childhood lymphangiomas with interferon-alpha. J Pediatr Hematol Oncol 1997;19:232-6.
GASTROINTESTINAL ENDOSCOPY
591