|Year : 2015 | Volume
| Issue : 3 | Page : 668
Nonconcomitant metachronous triple mesenchymal benign tumors in a child: Report of a case
Munisamy Ragavan1, Anbazhagan Duraiprabhu1, Eapen Koshy2, Selvendran Sugandhan3, Chakravarthy Srinivasan4, Janarthanam Sarvavinothini5
1 Department of Pediatric Surgery, MIOT International Hospital, Manapakkam, Chennai, Tamil Nadu, India
2 Department of Craniofacial Surgery, MIOT International Hospital, Manapakkam, Chennai, Tamil Nadu, India
3 Department of Dermatology, MIOT International Hospital, Manapakkam, Chennai, Tamil Nadu, India
4 Department of Pathology, MIOT International Hospital, Manapakkam, Chennai, Tamil Nadu, India
5 Department of Anesthesiology, MIOT International Hospital, Manapakkam, Chennai, Tamil Nadu, India
|Date of Web Publication||9-Oct-2015|
Department of Pediatric Surgery, MIOT International Hospital, Manapakkam 600 089, Chennai, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Two or more primary neoplastic lesions are very rare in children. We report a child who is first of its kind in English literature who presented with noncontiguous metachronous lipoma, chondroma, and fibrolipoma.
Keywords: Chondroma, fibrolipoma, lipoma, mesenchymal tumor
|How to cite this article:|
Ragavan M, Duraiprabhu A, Koshy E, Sugandhan S, Srinivasan C, Sarvavinothini J. Nonconcomitant metachronous triple mesenchymal benign tumors in a child: Report of a case. J Can Res Ther 2015;11:668
|How to cite this URL:|
Ragavan M, Duraiprabhu A, Koshy E, Sugandhan S, Srinivasan C, Sarvavinothini J. Nonconcomitant metachronous triple mesenchymal benign tumors in a child: Report of a case. J Can Res Ther [serial online] 2015 [cited 2020 Jul 14];11:668. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/668/140756
| > Introduction|| |
Two or more primary neoplastic lesions are very rare in children. Neoplastic lesions at an unusually young age, multifocality, bilateral lesion in paired organs and development of more than one primary tumor is suggestive of inherited predisposition to cancer. We present a case with nonconcomitant metachronous lipoma, chondroma, and fibrolipoma.
| > Case report|| |
A 6-year boy from Congo presented with slow growing painless warty skin masses in the torso since birth. His mother also noticed a lump in the left supraclavicular region since 1 year of age, which was increasing in size. A year back, there was onset of another lump below the left scapula, which is rapidly increasing in size. There is no history of pain, trauma or systemic symptoms. There is no family history of neoplastic disorder. Examination showed well-defined multiple, warty lesions each measuring 2-3 cm in the left scapular region altogether measuring 10 cm × 10 cm [Figure 1]. There was no tenderness, ulceration or fixity. Dermatologist made a diagnosis of nevus lipomatosus cutaneous superficialis. The supraclavicular lump was measuring 6 cm × 6 cm, hard and fixed with no neurovascular deficit. Possibility of bony tumor or sarcoma was thought off. The lump situated below the angle of left scapula was measuring 8 cm × 8 cm, soft and freely mobile deep to the muscles and hence diagnosed as deep seated soft tissue tumor. There was no neurocutaneous markers and regional lymphadenopathy. Systemic examination and routine laboratory examination was normal. Magnetic resonance imaging scan of the chest and neck showed verrucous dermal lesion which was free from subcutaneous fat. The supraclavicular mass was abutting subclavian vessels and brachial plexus with bony erosions and altered signals consistent with liposarcoma [Figure 2]. The infrascapular mass was suggestive of lipoma in the intermuscular plane. All these masses were confined to the left side of the spine and free from each other. Aspiration cytology of the lesions was done. There was gritty feeling during passage of the needle into the supraclavicular lesion, and the yield was poor. Aspirate from other two lesions showed fat laden cells with no features of malignancy. Parents wanted surgical excision of all the lesions in a single stage, and we explained the possibility of malignancy, need for postoperative multimodal therapy.
|Figure 1: Multiple warty nevus lipomatosus lesions and two separate lump in the left supraclavicular and infra scapular region|
Click here to view
|Figure 2: Magnetic resonance imaging scan of the chest and neck showing the verrucous dermal lesion, which was free from the subcutaneous fat and supraclavicular mass abutting subclavian vessels with bony erosions consistent with liposarcoma and infrascapular lipoma|
Click here to view
We planned excision through a large elliptical incision accessing all three lesions, with frozen biopsy back up. Patient in the supine position with head elevation and turned to the other side, an elliptical incision from the sternoclavicular joint was made. Supraclavicular mass was mobilized and excised in two pieces as it was adherent to the rib and access was difficult. The wound was closed partly and the patient was positioned prone. The incision was extended encircling the warty lesion and up to the infra scapular lesion. The warty lesion was excised in toto except one island of lesion, which was saved off from skin and the raw area left behind for secondary healing. The infra scapular lesion was enucleated. The frozen biopsy was suggestive of benign tumors. Wound was closed primarily with a suction drain, and it healed well [Figure 3]. Biopsy of the warty specimen showed a normal epidermis with the proliferation of mature adipocytes in the reticular dermis extending to the papillary dermis with small aggregates around the blood vessels and eccrine glands and a diagnosis of fibrolipoma was made [Figure 4]a. The supraclavicular masse showed chondrocytes similar to hyaline cartilage with large or double nuclei and foci of myxoid changes consistent with chondroma [Figure 4]b and the infrascapular mass showed mature adipocytes with fibrous capsule consistent with lipoma [Figure 4]c. The fat cells in the lesions showed no features of brown fat cells. By follow-up telephonic conversation he is doing well. After 6 months follow-up, he has no local recurrence but had hypertrophied scar in the back which responded partly to conservative treatment.
|Figure 3: Masses excised in to by an elliptical incision and healed scar with a raw area|
Click here to view
|Figure 4: High-power magnification (×40) of H and E stained biopsy specimen shows (a) fibrolipoma, (b) chondroma and (c) lipoma|
Click here to view
| > Discussion|| |
Synchronous or metachronous multiple tumors are rare, but it is well-described in adult cancer patients. Multiple primary cancers is said to be present if definite pattern of distinct malignant disease is present in two or more sites or tissues and is neither an extension, nor a recurrence, nor a metastasis of the primary lesion.  In a study, two primary malignancies are encountered in 3-5% and triple in 0.5%, quadruple in 0.3% of case.  Cancer patients have a 20% higher risk of new primary cancer, in the same organ or in another organ.  Factors responsible for multiple cancers remain largely unknown but, it may be attributable to prior shared etiologic factors, environmental exposures and inherited predisposition and prior cancer treatment.  There are few case reports of two benign tumors in children especially in the head and neck area and no case report of three benign tumors and one being congenital as in our case. There are reports of multiple soft tissue tumors due to the genetic condition like Bannayan-Riley-Ruvalcaba syndrome, Beckwith-Wiedemann syndrome, etc. , Multiple endocrine neoplasia syndrome is a well-described condition characterized by multiple endocrine and soft tissue tumors due genetic mutation.  The tumors that described on our patient does not fit into any described genetic conditions.
The most common benign tumors of the mesenchyme are the lipomas, which can occur anywhere in the body and can differentiate into a diversity of mesenchymal elements, such as blood vessels (angiolipoma), fibrous tissue (fibrolipoma), or muscle (myolipoma).  This concept of multilineage differentiation of pluripotent mesenchymal stem cells suggests that there are common predisposition or genetic defect and stem cell origin for multiple tumors. In our case, though the tumors are of different histology, but they are of the mesenchymal type and were located in the close proximity. This prompts us to think in terms of common etiology and may be due to genetic predisposition as one of the lesions was congenital.
Adipocytes give rise to white fat cells and brown fat cells. The brown fat which plays a role in preventing hypothermia in infants is distributed in the shoulders, neck and scapular region corresponding to the location of the tumors in our case. It was believed that after infants grow up, most of the mitochondria in brown adipose tissue disappear and becomes white fat. In rare cases, brown fat continues to grow and leads to a tumor called hibernoma.  In our case, the fat cells were mature type and one tumor was congenital hence, the possibility of hibernoma was ruled out.
| > Conclusion|| |
Multiple benign tumors in pediatric patients are extremely rare, and this case report describes the successful management of the first documented case of metachronous triple benign tumor. The exact cause could not be ascertained and may be due to a genetic problem causing mesenchymal stem cell differentiation disorder and neoplastic predisposition.
| > References|| |
Warren S, Gates O. Multiple primary malignant tumors. Am J Cancer 1932;16:1358-414.
Nemeth Z, Czigner J, Ivan L, Ujpal M, Barabas J, Szabo G. A case of quadruple cancer, including triple cancers in the head and neck region. J Oral Maxillofac Surg 1996;54:501-5.
Soerjomataram I, Coebergh JW. Epidemiology of multiple primary cancers. Methods Mol Biol 2009;471:85-105.
Travis LB, Hill D, Dores GM, Gospodarowicz M, van Leeuwen FE, Holowaty E, et al
. Cumulative absolute breast cancer risk for young women treated for Hodgkin lymphoma. J Natl Cancer Inst 2005;97:1428-37.
Soulard R, Nguyen AT, Souraud JB, Oddon PA, Fouet B, Cathelinaud O. Osteochondrolipoma of the submandibular region: A case report and review of the literature. Head Neck Pathol 2012;6:486-91.
Furlong MA, Fanburg-Smith JC, Miettinen M. The morphologic spectrum of hibernoma: A clinicopathologic study of 170 cases. Am J Surg Pathol 2001;25:809-14.
Bannayan GA. Lipomatosis, angiomatosis, and macrencephalia. A previously undescribed congenital syndrome. Arch Pathol 1971;92:1-5.
Beckwith JB. Macroglossia, omphalocele, adrenal cytomegaly, gigantism, and hyperplastic visceromegaly. Birth Defects Orig Artic Ser 1969;5:188-96.
Carney JA. Familial multiple endocrine neoplasia syndromes: Components, classification, and nomenclature. J Intern Med 1998;243:425-32.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]