Fibrolipoma on upper eyelid in child

oc000040 10.3205/oc000040 urn:nbn:de:0183-oc0000402 Case Report Fibrolipoma on upper eyelid in child Corredor-Osorio Corredor-Osorio Rafael R MD

Instituto Venezolano de Oftalmología, Av. Los Leones, CC Ciudad Paris, piso 2, Barquisimeto (Lara), Calle 137 casa 106-80B, Urb. Prebo 1, Valencia (Carabobo), Zone Code 2001, VenezuelaInstituto Venezolano de Oftalmología, Barquisimeto (Lara), Venezuela

raficorredor@yahoo.com author Ramos-Pineda Ramos-Pineda Nelly N

Instituto Venezolano de Oftalmología, Barquisimeto (Lara), Venezuela

author Eugenia Orellana Eugenia Orellana María M

Ocular Pathology, Section Pathology Institute Dr. Jose A. O’Daly, Universidad Central de Venezuela, Caracas, Venezuela

author German Medical Science GMS Publishing House

Düsseldorf

610 fibrolipoma lipoma benign tumor 20160317 engl This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). 2193-1496 6 GMS Ophthalmology Cases GMS Ophthalmology Cases 03 An 18-months-old male infant presented with a rapidly growing tumor on the right upper eyelid. Orbital computed tomography (CT) revealed a large, well-circumscribed mass with low density signal in the right upper eyelid. Magnetic resonance images (MRI) showed a lesion of mixed T1-signal intensity and high signal intensity in T2-weighted images. The tumor was treated by simple anterior orbitotomy with excisional biopsy, and the diagnosis of fibrolipoma was made by histopathologic examination. There was no evidence of tumor at the four-year follow-up. Fibrolipoma is one of the rare variant of the lipoma and only four cases have been reported in the orbit including the present case. Except for this case all other cases were reported in adults. IntroductionLipomas are common benign soft tissue neoplasms of mature adipose tissue , . Although fat is abundant within the orbit, orbital lipomas are rare . A fibrolipoma is an extremely rare subtype of lipomas from connective tissue tumors composed of mature adipocytes, which are commonly benign . Fibrolipomas belong to the family of fat-containing lesions and are benign tumor characterized by the presence of adipose tissue and abundant amounts of fibrous tissues , , . They are well-separated from the surrounding tissues and usually occur in adults . Three cases of fibrolipoma of the orbit in adults have been reported. We report another case of the fibrolipoma of the upper eyelid in an 18-months-old male infant. Case descriptionAn 18-months-old male developed a tumor on his right upper eyelid over the course of two months. The tumor became significantly prominent, progressive enlargement over the last month. There was history of trauma to the area. On physical examination he was found to have a very large tumor, approximately 2×2 cm on the right upper eyelid, there appeared to be no discolorations, erythema, or signs of inflammation, it was a firm mass, and nontender to the palpation (Figure 1 ). The remaining ocular examination was normal. The patient had no systemic manifestations. Orbital CT images demonstrated a large, well-circumscribed mass with a low density signal similar to intraorbital fat on the right upper eyelid. MRI presented a lesion with high signal intensity in T2-weighted images, and the lesion showed a well-marginated soft tissue mass with mixed signal intensity in T1-weighted images (Figure 2 </PlainText></TextGroup><ImgLink imgNo="2" imgType="figure"/>). The superior visual field was compromised secondary to mechanical ptosis. The patient underwent an uncomplicated anterior orbitotomy through a superior eyelid crease incision and excision of the tumor (<TextGroup><PlainText>Figure 3 </PlainText></TextGroup><ImgLink imgNo="3" imgType="figure"/>). The histological examination of the specimen revealed a lesion composed of mature adipose lobes separated by thick dense connective tissue tracts providing the diagnosis of a fibrolipoma (Figure 4 <ImgLink imgNo="4" imgType="figure"/>). At follow-up four years postoperatively the cosmetic appearance of the eyelid was very satisfactory and there had been no recurrence of the tumor.</Pgraph></TextBlock> <TextBlock linked="yes" name="Discussion"> <MainHeadline>Discussion</MainHeadline><Pgraph>Lipomas are well-circumscribed slow growing, benign tumors composed of mature fat cell grouped in lobules by connective tissue septa <TextLink reference="6"></TextLink>. These tumors are very rare in the orbit, accounting for less than 1% of all orbital tumors <TextLink reference="7"></TextLink>. The pooled incidence of lipoma was 0.6% in a large orbital tumor series <TextLink reference="3"></TextLink> and 1.6% of facial lipomas <TextLink reference="4"></TextLink>. Fibrolipoma is a benign tumor that rarely occurs in the orbit, and is classified as a variant of conventional lipoma by the World Health Organization (WHO), because present components of mature adipose cell and bands of dense connective tissue <TextLink reference="3"></TextLink>, <TextLink reference="6"></TextLink>, <TextLink reference="8"></TextLink>. They occur mostly in adult men <TextLink reference="4"></TextLink>. The age of the patient in this case was unusual, because these lesions peak incidence is in the fifth and sixth decade of life <TextLink reference="1"></TextLink>, <TextLink reference="6"></TextLink>, and they are rarely observed under the age of 20 years <TextLink reference="6"></TextLink>. The clinical features of fibrolipoma vary according to their rate of growth, size and location. Clinically, fibrolipoma usually presents as an asymptomatic, slowly growing mass, firm or soft consistency <TextLink reference="4"></TextLink> and well circumscribed <TextLink reference="6"></TextLink>. Occurs not only in subcutaneous tissue, but also in various areas such as the oral cavity, trachea, esophagus, parotid gland, spermatic cord <TextLink reference="4"></TextLink>, <TextLink reference="6"></TextLink>, larynx, pharynx, colon <TextLink reference="6"></TextLink>, <TextLink reference="9"></TextLink> and nose <TextLink reference="4"></TextLink>. According to the literature, it is difficult to value the real incidence of this neoplasm because it appears as painless and slow-growing clinical appearance. In reality, the patient refers to the clinician only when it becomes symptomatic and for functional problems. The size of tumor depends on the location of the lesion. Most of the lesions are less than 3 cm in size, but occasionally lesions become much larger. The consistency of this lesion varies from soft to firm, depending on quantity and distribution of fibrous tissue and depth of the tumor <TextLink reference="1"></TextLink>, <TextLink reference="9"></TextLink>. A characteristic feature is a change in consistency and form of many of these tumors during contraction of involved muscle. The tumor is soft and flat when the muscle is relaxed and becomes firm and more spherical when muscle contracts <TextLink reference="2"></TextLink>. MRI is very useful for the diagnosis of all kinds of lipomas. Tumors have high signal intensity on T1-weighted images, with relative decreasing signal on T2-weighted images. A fat suppressed MRI is particularly beneficial for diagnosis. </Pgraph><Pgraph>Fibrolipomas are more heterogeneous than lipomas on MRI images. On CT images, lipomas can be seen as an uncontrasted hypodense mass <TextLink reference="6"></TextLink>.</Pgraph><Pgraph>The exact etiology of lipomas and their variants is not well established. An association with genetic aberrations has been described for most types of adipocytic tumor and some trauma mechanisms, such as origin from lipoblastic embryonic cell nest, blunt trauma or chronic intermittent compression, have been associated with the apparition of lipomas <TextLink reference="10"></TextLink>. Fatty degeneration, hormonal basis, infection and infarction, metaplasia of muscle cells and chronic irritation are probable representative theories to elucidate the pattern of lipoma <TextLink reference="1"></TextLink>, <TextLink reference="2"></TextLink>. Fibrolipoma has been thought to be congenital to be caused by endocrinal imbalance, to be the product of a degenerated fibromatous tumor, or to arise from maturation of lipoblastomatosis <TextLink reference="2"></TextLink>. In our case, the patient had no systemic disease, and he did not have any history of trauma, neither family history of fibrolipoma. Histologically lipomas are classified as a simple lipoma or variant as angiolipoma, chondroid lipoma, myolipoma, spindle cell, pleomorphic lipoma, diffuse lipomatous proliferations (lipomatosis) and hibernoma <TextLink reference="2"></TextLink>, <TextLink reference="6"></TextLink>, <TextLink reference="8"></TextLink>. Some of these show distinctive clinico-pathological features that are usually discernible only after histological examination <TextLink reference="8"></TextLink>. Histologically, when there is a proliferation of components of mature adipose surrounded by dense fibrous cell connective tissue, the tumor is labeled a fibrolipoma <TextLink reference="1"></TextLink>, <TextLink reference="3"></TextLink>, <TextLink reference="4"></TextLink>. Additionally, fibrolipomas have a higher proliferative activity than the classic variants <TextLink reference="6"></TextLink>, <TextLink reference="9"></TextLink> but no differences in clinical behavior were noticed after surgical treatment <TextLink reference="2"></TextLink>. Although fibrolipomas are benign tumors, there are few cases of conversion to liposarcoma in the literature <TextLink reference="6"></TextLink>. Fibrolipoma should be differentiated from the other microscopic variants. The angiolipoma consists of an admixture of mature fat and numerous small blood vessels <TextLink reference="10"></TextLink>, and usually affect male adolescents <TextLink reference="11"></TextLink>. Myxoid lipoma microscopally, these lipomas were well-circumscribed and contained adipocytes of variable size and myxoid areas too, exhibits a mucoid background and may be confused with myxoid liposarcoma <TextLink reference="11"></TextLink>. The spindle cell lipoma demonstrates a variable amount of uniform appearing spindle cells in conjunction with a more typical lipomatous component. Pleomorphic lipomas are characterized by presence of spindle cells and bizarre hyperchromatic giant cells. Intramuscular lipomas are often more deeply situated and have an infiltrative growth pattern that extends between skeletal muscle bundles <TextLink reference="1"></TextLink>. The clinical differential diagnosis considered for the present case includes epidermoid cyst and dermoid cyst. </Pgraph><Pgraph>Because of the diverse modes of presentation, some other lesions should be considered in the clinical differential diagnosis and these include fibrous histiocytoma, fasciitis nodular and schawannoma. Definitive diagnosis can be based on histological appearance. The treatment of fibrolipoma, including all the histological variants is complete surgical excision. In our case the patient requested the removal of his lesion because of concerns rapidly growing, and the esthetic even so functional aspects resulting from the compression of focal structures. The most common symptom described was rapidly growing painless swelling. Medical management of lipomas includes steroid injections (triamcinolone acetonide) that result in local fat atrophy, thus, shrinking the tumor size. They are best done on lipomas that are less than <TextGroup><PlainText>2.54 cm</PlainText></TextGroup> in diameter. Average volume of steroid used may range from 1 to 3 ml depending on the size of tumor <TextLink reference="12"></TextLink>. In summary, fibrolipoma is one rare variant of the lipoma. In the present case, the treatment of choice was a complete resection of the tumor. Due to the similarity of clinical pictures of all lipomas, the histopathological examination of the excised tissue is the gold standard for diagnosis. The prognosis of fibrolipoma is excellent and recurrence is rare. Fibrolipoma should be included in the differential diagnosis when a localized mass in the eyelid is encountered. Therefore it is essential for ophthalmologists to be familiarized with these lesions. The case presented here adds to the existent 3 cases of fibrolipoma orbit reported in the literature.</Pgraph></TextBlock> <TextBlock linked="yes" name="Notes"> <MainHeadline>Notes</MainHeadline><SubHeadline>Competing interests</SubHeadline><Pgraph>The authors declare that they have no competing interests.</Pgraph></TextBlock> <References linked="yes"> <Reference refNo="1"> <RefAuthor>Khubchandani M</RefAuthor> <RefAuthor>Thosar NR</RefAuthor> <RefAuthor>Bahadure RN</RefAuthor> <RefAuthor>Baliga MS</RefAuthor> <RefAuthor>Gaikwad RN</RefAuthor> <RefTitle>Fibrolipoma of buccal mucosa</RefTitle> <RefYear>2012</RefYear> <RefJournal>Contemp Clin Dent</RefJournal> <RefPage>S112-4</RefPage> <RefTotal>Khubchandani M, Thosar NR, Bahadure RN, Baliga MS, Gaikwad RN. Fibrolipoma of buccal mucosa. Contemp Clin Dent. 2012 Apr;3(Suppl 1):S112-4. 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Note that overlying skin is not erythematous, edematous or discolored.</Mark1></Pgraph></Caption> </Figure> <Figure format="png" height="691" width="447"> <MediaNo>2</MediaNo> <MediaID>2</MediaID> <Caption><Pgraph><Mark1>Figure 2: CT images show a 7.4×23.3 mm-sized low density mass in the upper eyelid of the right orbit (A). T2-weighted magnetic resonance imaging (MRI) presenting a high signal intensity (B).</Mark1></Pgraph></Caption> </Figure> <Figure format="png" height="299" width="451"> <MediaNo>3</MediaNo> <MediaID>3</MediaID> <Caption><Pgraph><Mark1>Figure 3: Peroperative photograph shows a yellowish-white soft tissue mass.</Mark1></Pgraph></Caption> </Figure> <Figure format="png" height="357" width="446"> <MediaNo>4</MediaNo> <MediaID>4</MediaID> <Caption><Pgraph><Mark1>Figure 4: Microscopy showed a lesion composed of mature adipose lobes separated by thick dense connective tissue tracts (H and E, x20).</Mark1></Pgraph></Caption> </Figure> <NoOfPictures>4</NoOfPictures> </Figures> <InlineFigures> <NoOfPictures>0</NoOfPictures> </InlineFigures> <Attachments> <NoOfAttachments>0</NoOfAttachments> </Attachments> </Media> </OrigData> </GmsArticle>