Cervical Lymphadenitis Due to Nodular Fasciitis in a Child

Address for correspondence: Dr. Kayı Eliaçık E-mail: kayieliacik@gmail.com 1Dr. Ali Kanik1, MD, Department of Paediatrics, 2Dr. Kayi Eliacik, MD, Department of Paediatrics, 3Dr. Tolga Ince, MD, Department of Paediatrics, 4Dr. Umit Bayol, MD, Associate Professor Department of Pathology, 5Dr. Mehmet Helvaci, MD, Associate Professor in Paediatrics. All from Tepecik Teaching and Research Hospital, Izmir, Turkey. Abstract


Introduction
N odular fascii s (NF) is a rare non-neoplas c so ssue lesion mainly composed of myofi broblas c cells and fi broblasts.It is also known as subcutaneous pseudosarcomatous fi bromatosis, infl ammatory pseudotumor or prolifera ve fascii s 1 .Although 10-15% of the cases have a history of trauma, most cases have no risk factors.In general, it is a rapidly growing self-limi ng solitary lesion.The diameters of the lesions have been reported to be 0.5-10 cm, most of them being smaller than 4 cm 1 .NF has been commonly seen in adults, with only 10 % of the cases being children 2,3 .NF has been extensively seen in the trunk and upper extremi es, but rarely in the head and neck region.Head and neck localiza on is more common in childhood than in adulthood 4 .NF is a self-limi ng nonneoplas c so ssue lesion, with no signifi cant gender preference.The diagnosis must be histopathologically confi rmed 2 .Excisional biopsy and surgical resec on are cura ve 3 .In this paper, we present a pediatric case who admi ed with symptoms of lymphadeni s and diagnosed as NF clinically and histopathologically.

The Case
A two-year-old girl referred to our clinic with complaints of fever and swelling on the right side of her neck.The fever and neck lump have started just a couple of days ago.The vital signs were normal except fever.Physical examina on revealed a 4x6 cm palpable packed lymph nodule on the right side of her neck.
Other systemic examina ons were within normal limits.Ini al tests showed hemoglobin of 11.2g/dl, leukocyte of 26,600/cu mm, and platelet of 4,53,000/ cu mm.Serum glucose, electrolytes, renal, hepa c func on tests, and serum immunoglobulin levels were all within normal limits.Erythrocyte sedimenta on rate was 75 mm/h, and C-reac ve protein was 85 mg/dl.Viral markers, serum markers for brucellosis and tuberculosis were nega ve.Nonspecifi c an biotherapy (sulbactam-ampicillin) was tried, however the cervical lump was unresponsive to an bio cs.The ultrasonography examina on showed a hypoechogenic and heterogenic mass at the right posterior

Discussion
Lymphadenopathy is the pathology of the lymph nodes as regards dimension, number and consistency.Lymphadenomegaly may be the ini al sign of a disease, or itself is the primary problem.The diff eren al diagnoses of lymphadenomegaly comprise infec ons, malignancies, immunologic distorbencies, Castleman's disease (angiofollicular lymph node hyperplasia), sinus his ocytosis, and infl ammatory pseudotumors.
Nodular fascii s is a so ssue lesion composed of prolifera ng fi broblasts.The lesion is described as pseudosarcomatous or prolifera ve fi bromatosis, demonstra ng an alarming nodular prolifera on of fi broblasts.This designa on is due to resemblance to sarcomas.It is a benign lesion, but it is some mes mistaken for fi brosarcoma 4 .Nodular fascii s is not common during childhood, with the cases typically presen ng with mass lesions in the head and neck region.Chris na J. et al. 5 found 39% of localiza on to be in the head and head neck region in an 18-case series of NF.Kim et al. 6 have reported two cases (16-month-old and 11-year-old) with periorbital and supraclavicular, rapidly growing up to 4.6 cm and 3.5 cm masses, respec vely.Though NF in the head and neck region is not very rare among the pediatric popula on, there is almost no cases presen ng as cervical lymphadeni s in the English literature.Many NF cases may be overlooked due to uncertain clinic symptoms and histopathological fi ndings 1 .
In NF, the lesion is typically submucosal, deeply oriented among the so ssue compartments and wellcircumscribed.It is nodular and tender on palpa on because of the strength of the bonds in submucosal ssue.It frequently grows rapidly without evidence of associated infec on and may present histologic fi ndings similar to those seen in malignant tumors, During the follow-up, the lump con nued to enlarge.Though total surgical excision was planned, because of the fi xed mass with surrounding ssues, mul ple core needle biopsies have been performed.The biopsy fi ndings were consistent with NF.An infl ammatory process consis ng of vascular prolifera on, mononuclear cells and fi broblas c prolifera on was disrup ng striated muscle and invading the lymph node (Fig. 2).She received no specifi c therapies and the lump disappeared within a month.The case has been followed up for the past 30 months and there has been no evidence of recurrence.such as high cellularity, increased mito c ac vity, and infi ltra ve growth pa ern 7 .Ozben et al. 7 reported a case of NF of the breast previously misdiagnosed as breast carcinoma.The key cytological features for diagnosis are plump, immature fi broblas c cells, chronic infl ammatory cells, capillary prolifera on, and vascular channels with extravasated red blood cells in an edematous myxoid background.There is mito c ac va on, with no atypical cells.Immunohistochemical examina on shows that spindle cells express vimen n and ac n, but not desmin, kera n or S-100 proteins 5,7 .Rapid onset, chronic infl amma on in the stroma, and the absence of atypia suggests that the lesion is benign 8 .In our case, vascularly rich fi brous and infl ammatory process was disrup ng striated muscle and involving the lymph node.Fibroblas c cells had oval and palestaining nuclei with no atypia.There were no atypical mito c features (Fig 3).
Neither fi ne-needle aspira on cytology nor core needle biopsy can easily diagnose NF because it is diffi cult to obtain representa ve cells to make an accurate diagnosis using these techniques.The diagnosis usually requires the histopathological examina on of an excisional biopsy 7 .An accurate histopathological diagnosis prevents an aggressive surgery.It has also been reported that the rest of the lesion disappeared spontaneously a er the diagnos c biopsy 1 .In our case, the lesion disappeared within a month.

Conclusion
We conclude that NF is a rare benign lesion during childhood.Lymphadenomegaly may be the ini al presenta on of NF, which should be included in the diff eren al diagnosis of superfi cial and deep so ssue masses of the neck, especially in recently developed rapidly growing masses.

Fig 1 :
Fig 1: A 4x6 cm conglomerate lymph nodule on the right side of the neck on MRI Fig.2c