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LYMPHANGIOMA OF TONGUE:
A RARE CASE REPORT
 INDIAN DENTAL ACADEMY
 Leader in continuing Dental
Education
www.indiandentalacademy.com
INTRODUCTION
Lymphangiomas are rare benign hamartomatous
lesions of the lymphatic system .
Virchow (1854) gave the first accurate description
of lymphangioma.
The Lymphangiomas are localized in the head and
neck area in about 75% of cases and about 80% of
these cases are children less than 2 years old.
In the oral cavity, lymphangioma is a rare, non-
odontogenic, benign neoplasm, which originates
from lymph vessels.
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About 40-50% of Lymphatic malformations are
occurs in the tongue, which is the preferred site
of intraoral involvement followed by buccal
mucosa which is the second most common site.
Although tongue is a rare site for lymphangioma
it is the most common cause for macroglossia.
In tongue it mostly occurs on the dorsal surface
and lateral border and rarely arises on the
palate, gingiva, buccal mucosa and lips. These
tumors rarely regress and they keep on growing
slowly.
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Lymphangioma simplex (capillary lymphangioma,
lymphangioma circumscriptum):- Composed of small, thin-
walled lymphatics.
Cavernous lymphangioma :- Comprised of dilated lymphatic
vessels.
Cystic lymphangioma (cystic hygroma) :- Consisting of huge,
macroscopic lymphatic spaces with surrounding fibrovascular
tissues and smooth muscle.
Lymphangioma complex:- Consist of lymphatic channels
appear to be dissecting through dense collagen bundles.
Lymphangioma can be classified into
four categories:-
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CASE REPORT
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Presence of enlarged tongue with multifocal, pebbly, vesicle like
lesions on the tip, dorsal and lateral surfaces of tongue. The color
of these lesions are ranging from white to yellowish. Lesions
having so called frog egg appearance. Tongue is having glossy
appearance with transverse fissures on the dorsal surface of
tongue.
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Differential diagnosis
Lymphangioma
Hemangioma on tongue
Vascular malformations
Cystic hygroma
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Investigations
Routine blood investigations showed all
values within normal range.
Cytological smear made from the tongue
and stained with PAP revealed presence of
desquamated epithelial cells which are
scattered and clumped at places.
No fungal elements were found.
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Occlusal radiograph showed no
abnormalities.
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Ultrasound
Ultrasound showed thickening of the tongue with
multiple, linear torturous vascular channels
mainly on the ventral part in the anterior half of
the tongue. Color doppler and power doppler
shows minimal blood flow in the vascular
channels.
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Final Diagnosis
On the basis of history, clinical examination
and investigations, patient was diagnosed
as a case of lymphangioma of tongue.
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DISCUSSION
Although no clear mechanism for lesion development
exists, the lesions likely develop as a result of primitive
embryonic theory. They consist of functionally blocked
lymphatic tracts. This blockage may result in increased
hydrostatic pressure with subsequent expansion of the
lesion until a pressure equilibrium is achieved with the
surrounding tissues. The importance of the surrounding
tissues in determining the nature of the lesion is evident,
as microcystic lesions are more common in the tongue,
whereas macrocystic lesions predominate in the
relatively compliant tissues of the neck.
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50% lymphatic malformations are present at birth, and
90% are diagnosed by the time the individual is aged 2
years. However, the time of diagnosis can range from 19
weeks' gestation to the individual's second decade of life.
Superficial lymphatic malformations have a pebbly
surface and are clear or bluish. The bluish color may be
caused by either a venous component or an intralesional
hemorrhage.
Deeper lesions may have a superficial component.
When a superficial component is absent, deeper lesions
usually appear as soft, ill-defined masses that markedly
distort the local anatomy.
Macrocystic lesions may be fluctuant and readily
transilluminating.
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Histologic Findings
Superficial lesions consist of dilated lymph vessels lined
by flat endothelial cells in a discontinuous layer.
Deeper lesions consist of irregular, dilated, and
interconnected lymphatic vessels, some of which reveal
macroscopic cyst formation. The walls of the vessels
variably contain smooth muscle bundles. Cystic spaces
generally contain a lightly eosinophilic, proteinaceous
fluid with scant lymphocytes.
In contrast to vascular malformations, electron
microscopy and immunohistochemical techniques
demonstrate irregular and fragmented basal lamina in
the lymphatic malformations.
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Imaging Studies
Approximately 40% of lesions are diagnosed on
the basis of their clinical appearance alone;
Imaging plays several roles in the evaluation
and treatment of oral lymphatic malformations.
In determining the extent of the lesion and its
proximity to vital structures.
In determining whether the lesion contains a
vascular component.
Is used to assess recurrence in treated
lesions.
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Complete surgical excision is treatment of choice but
this is not always possible as when there is
extensions into base tongue, floor of mouth, larynx
and neurovascular structures of neck. Recurrent,
residual or surgically challenging tumors are treated
with intralesional injection of sclerosing agents like
25% dextrose, hypertonic saline, bleomycin, OK-432
(picibanil) before surgery. A change in consistency
of the tumor, manifests by softening, is followed by
marked shrinkage. Satisfactory results can be
obtained, resulting in definite reduction in size and
improvement in cosmetic appearance.
www.indiandentalacademy.com
In recent years Carbon dioxide and
Neodynium Yitrium Aluminum Garnet (Nd-
YAG) laser photocoagulation surgery has
become popular. Earlier steroids,
electrocoagulation, cryotherapy or
radiation therapy has been used with
variable results but most effective
treatment is surgery .
No appropriate medical care is available
for oral lymphatic malformations, other
than observation in selected patients.
www.indiandentalacademy.com
REFERENCES
 Extensive Lymphangioma Presenting With Upper Airway
Obstruction -Arch Otolaryngol Head Neck Surg. 2000;126:1378-
1382.
 Lymphangioma : A Tongue in Cheek Situation BMJ, 2004;
329 : 1386.
 Text book of Oral Medicine by Dr Anil Govindrao Ghom 1st
ed.
 Sidle DM, Maddalozzo J, Meier JD, et al. Altered pigment
epithelium-derived factor and vascular endothelial growth factor
levels in lymphangioma pathogenesis and clinical
recurrence. Arch Otolaryngol Head Neck
Surg. Nov 2005;131(11):990-5. [Medline].
 Sabin FR. The lymphatic system in human embryos, with a
consideration of the morphology of the system as a whole. Am J
Anat. 1909;9:43-91.
 Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25
years' experience with lymphangiomas in children. J Pediatr
Surg. Jul 1999;34(7):1164-8. [Medline].
 Edwards PD, Rahbar R, Ferraro NF, Burrows PE, Mulliken
JB. Lymphatic malformation of the lingual base and oral
floor. Plast Reconstr Surg. Jun 2005;115(7):1906-15. [Medline].
www.indiandentalacademy.com
Neville BW, Damm DD, Allen CM, Bouquot JE: Soft
Tissue Tumors (2002). In Oral and Maxillofacial
pathology 2nd edition. Edited by: Neville BW.
Philadelphia: W.B. Saunders; 475-7.
Jeeva Rathan J, Harsha Vardhan BG, Muthu MS, V,
Saraswathy K, Sivakumar N. Oral lymphangioma: A
case report. J Indian Soc Pedod Prev Dent [serial online]
2005 [cited 2008 Mar 15];23:185-9.
Pediatr Emerg Care. 1995 Jun;11(3):183-5
The Internet Journal of Otorhinolaryngology TM
ISSN: 1528-8420
Journal of Indian Society of Pedodontics and Preventive
Dentistry Year : 2005 | Volume : 23 | Issue : 4 |
Page : 185-189
www.indiandentalacademy.com
THANK-YOUTHANK-YOU
www.indiandentalacademy.com

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Lymphangioma of tongue/ dental courses

  • 1. LYMPHANGIOMA OF TONGUE: A RARE CASE REPORT  INDIAN DENTAL ACADEMY  Leader in continuing Dental Education www.indiandentalacademy.com
  • 2. INTRODUCTION Lymphangiomas are rare benign hamartomatous lesions of the lymphatic system . Virchow (1854) gave the first accurate description of lymphangioma. The Lymphangiomas are localized in the head and neck area in about 75% of cases and about 80% of these cases are children less than 2 years old. In the oral cavity, lymphangioma is a rare, non- odontogenic, benign neoplasm, which originates from lymph vessels. www.indiandentalacademy.com
  • 3. About 40-50% of Lymphatic malformations are occurs in the tongue, which is the preferred site of intraoral involvement followed by buccal mucosa which is the second most common site. Although tongue is a rare site for lymphangioma it is the most common cause for macroglossia. In tongue it mostly occurs on the dorsal surface and lateral border and rarely arises on the palate, gingiva, buccal mucosa and lips. These tumors rarely regress and they keep on growing slowly. www.indiandentalacademy.com
  • 4. Lymphangioma simplex (capillary lymphangioma, lymphangioma circumscriptum):- Composed of small, thin- walled lymphatics. Cavernous lymphangioma :- Comprised of dilated lymphatic vessels. Cystic lymphangioma (cystic hygroma) :- Consisting of huge, macroscopic lymphatic spaces with surrounding fibrovascular tissues and smooth muscle. Lymphangioma complex:- Consist of lymphatic channels appear to be dissecting through dense collagen bundles. Lymphangioma can be classified into four categories:- www.indiandentalacademy.com
  • 6. Presence of enlarged tongue with multifocal, pebbly, vesicle like lesions on the tip, dorsal and lateral surfaces of tongue. The color of these lesions are ranging from white to yellowish. Lesions having so called frog egg appearance. Tongue is having glossy appearance with transverse fissures on the dorsal surface of tongue. www.indiandentalacademy.com
  • 7. Differential diagnosis Lymphangioma Hemangioma on tongue Vascular malformations Cystic hygroma www.indiandentalacademy.com
  • 8. Investigations Routine blood investigations showed all values within normal range. Cytological smear made from the tongue and stained with PAP revealed presence of desquamated epithelial cells which are scattered and clumped at places. No fungal elements were found. www.indiandentalacademy.com
  • 9. Occlusal radiograph showed no abnormalities. www.indiandentalacademy.com
  • 10. Ultrasound Ultrasound showed thickening of the tongue with multiple, linear torturous vascular channels mainly on the ventral part in the anterior half of the tongue. Color doppler and power doppler shows minimal blood flow in the vascular channels. www.indiandentalacademy.com
  • 11. Final Diagnosis On the basis of history, clinical examination and investigations, patient was diagnosed as a case of lymphangioma of tongue. www.indiandentalacademy.com
  • 12. DISCUSSION Although no clear mechanism for lesion development exists, the lesions likely develop as a result of primitive embryonic theory. They consist of functionally blocked lymphatic tracts. This blockage may result in increased hydrostatic pressure with subsequent expansion of the lesion until a pressure equilibrium is achieved with the surrounding tissues. The importance of the surrounding tissues in determining the nature of the lesion is evident, as microcystic lesions are more common in the tongue, whereas macrocystic lesions predominate in the relatively compliant tissues of the neck. www.indiandentalacademy.com
  • 13. 50% lymphatic malformations are present at birth, and 90% are diagnosed by the time the individual is aged 2 years. However, the time of diagnosis can range from 19 weeks' gestation to the individual's second decade of life. Superficial lymphatic malformations have a pebbly surface and are clear or bluish. The bluish color may be caused by either a venous component or an intralesional hemorrhage. Deeper lesions may have a superficial component. When a superficial component is absent, deeper lesions usually appear as soft, ill-defined masses that markedly distort the local anatomy. Macrocystic lesions may be fluctuant and readily transilluminating. www.indiandentalacademy.com
  • 14. Histologic Findings Superficial lesions consist of dilated lymph vessels lined by flat endothelial cells in a discontinuous layer. Deeper lesions consist of irregular, dilated, and interconnected lymphatic vessels, some of which reveal macroscopic cyst formation. The walls of the vessels variably contain smooth muscle bundles. Cystic spaces generally contain a lightly eosinophilic, proteinaceous fluid with scant lymphocytes. In contrast to vascular malformations, electron microscopy and immunohistochemical techniques demonstrate irregular and fragmented basal lamina in the lymphatic malformations. www.indiandentalacademy.com
  • 15. Imaging Studies Approximately 40% of lesions are diagnosed on the basis of their clinical appearance alone; Imaging plays several roles in the evaluation and treatment of oral lymphatic malformations. In determining the extent of the lesion and its proximity to vital structures. In determining whether the lesion contains a vascular component. Is used to assess recurrence in treated lesions. www.indiandentalacademy.com
  • 16. Complete surgical excision is treatment of choice but this is not always possible as when there is extensions into base tongue, floor of mouth, larynx and neurovascular structures of neck. Recurrent, residual or surgically challenging tumors are treated with intralesional injection of sclerosing agents like 25% dextrose, hypertonic saline, bleomycin, OK-432 (picibanil) before surgery. A change in consistency of the tumor, manifests by softening, is followed by marked shrinkage. Satisfactory results can be obtained, resulting in definite reduction in size and improvement in cosmetic appearance. www.indiandentalacademy.com
  • 17. In recent years Carbon dioxide and Neodynium Yitrium Aluminum Garnet (Nd- YAG) laser photocoagulation surgery has become popular. Earlier steroids, electrocoagulation, cryotherapy or radiation therapy has been used with variable results but most effective treatment is surgery . No appropriate medical care is available for oral lymphatic malformations, other than observation in selected patients. www.indiandentalacademy.com
  • 18. REFERENCES  Extensive Lymphangioma Presenting With Upper Airway Obstruction -Arch Otolaryngol Head Neck Surg. 2000;126:1378- 1382.  Lymphangioma : A Tongue in Cheek Situation BMJ, 2004; 329 : 1386.  Text book of Oral Medicine by Dr Anil Govindrao Ghom 1st ed.  Sidle DM, Maddalozzo J, Meier JD, et al. Altered pigment epithelium-derived factor and vascular endothelial growth factor levels in lymphangioma pathogenesis and clinical recurrence. Arch Otolaryngol Head Neck Surg. Nov 2005;131(11):990-5. [Medline].  Sabin FR. The lymphatic system in human embryos, with a consideration of the morphology of the system as a whole. Am J Anat. 1909;9:43-91.  Alqahtani A, Nguyen LT, Flageole H, Shaw K, Laberge JM. 25 years' experience with lymphangiomas in children. J Pediatr Surg. Jul 1999;34(7):1164-8. [Medline].  Edwards PD, Rahbar R, Ferraro NF, Burrows PE, Mulliken JB. Lymphatic malformation of the lingual base and oral floor. Plast Reconstr Surg. Jun 2005;115(7):1906-15. [Medline]. www.indiandentalacademy.com
  • 19. Neville BW, Damm DD, Allen CM, Bouquot JE: Soft Tissue Tumors (2002). In Oral and Maxillofacial pathology 2nd edition. Edited by: Neville BW. Philadelphia: W.B. Saunders; 475-7. Jeeva Rathan J, Harsha Vardhan BG, Muthu MS, V, Saraswathy K, Sivakumar N. Oral lymphangioma: A case report. J Indian Soc Pedod Prev Dent [serial online] 2005 [cited 2008 Mar 15];23:185-9. Pediatr Emerg Care. 1995 Jun;11(3):183-5 The Internet Journal of Otorhinolaryngology TM ISSN: 1528-8420 Journal of Indian Society of Pedodontics and Preventive Dentistry Year : 2005 | Volume : 23 | Issue : 4 | Page : 185-189 www.indiandentalacademy.com