Search :     
Cases
1999-12-17-05 Answer of case of the week #15 © Clavelli www.TheFetus.net

Answer to case #15

December 17, 1999- January 7, 2000

Submitted and discussed by Adrian Clavelli, Buenos Aires, Argentina, Dr. Brosto and Philippe Jeanty, MD, PhD

These are images from a midtrimester fetus with something behind the head 

  

 

These images ARE NOT sufficient to make the diagnosis. There is also a 2.7 MB video available

Findings

The images demonstrated a soft-tissue mass on the postero-lateral aspect of the head. The cranium was intact and the brain architecture unremarkable. There is a little molding of the skull at the level of the mass, but not very significant.

A few other characteristics of the mass were important to notice:

1)          it was mostly homogeneous

2)          it has a fair amount of vascular flow

3)          it is not cystic

4)          it contains some small echogenicities which could represent calcifications (phleboliths).

Differential diagnosis

The differential diagnosis of soft-tissue masses (including soft-tissues tumors of the limbs) includes (most common are boldface):

1)          angiofibroma

2)          cystic lymphangioma [1] , [2]

3)          desmoid fibromatosis

4)          fibromatosis colli [3]

5)          fibrosarcoma [4] , [5] , [6] , [7] , [8]

6)          fibrous hamartoma of infancy

7)          fibrous histiocytomas [9]

8)          hemangiomas [10] , [11]

9)          hemangiopericytoma [12] , [13]

10)     hyaline fibromatosis [14]

11)     lipoblastomatosis [15] , [16]

12)     lymphangioma

13)     lymphangiomatosis [17]

14)     myofibromatosis [18] , [19] , [20]

15)     neurofibromas [21]

16)     rhabdomyoma [22]

17)     soft tissue sarcomas

18)     subcutaneous fat necrosis [23]

19)     teratoma [24]

This is a fairly large differential diagnosis of uncommon tumors !

In a review [25] of 190 soft tissue tumors in infants between birth and 12 months of age, 75% of cases were benign with the majority of cases being hemangioendothelioma (64% including 32% of capillary hemangioma), lymphangioma (29%) [26] , and fibromatosis-myofibromatosis. Fibrous histiocytoma and lipoblastoma were the next two less common lesions. Infantile fibrosarcoma is considered a borderline tumor (it can metastasis, but do so rarely). The embryonal rhabdomyosarcoma and peripheral primitive neuroectodermal tumor are the most common malignant soft tissue tumors.

The characteristic of soft tissue tumors in the first year of life are slightly different from those of older age group:

  1. They are more often benign than those of later childhood despite their cellularity and presence of mitotic activity.
  2. Fibroblastic-myofibroblastic tumors are more frequent in the first year of life, whereas neurogenic and myogenic tumors are relatively more common in children older than 1 year of age.
  3. There is a predisposition for the trunk, head and neck region whereas the extremities are mostly affected in children beyond the first year of life.

In another large study, lymphangiomas were most frequent lesions of vascular origin during the first year of life, most commonly arising on the head, neck and axilla [27] . 

Although teratoma (see also teratoma with hydrops) was a popular answer, these lesions are usually more antero-lateral and disorganized than what was present in the current case. These solid masses are generally unilateral and encapsulated, they vary in size and generally consist in mixture of cystic and solid components, calcifications can be detected in 50 % of the cases [28] . Some of the images demonstrate small hyperechoic spots that could be calcifications.

 Hemangiomas are soft-tissue tumors that have the same appearance and are the most common tumors solid found in fetuses. This was the preliminary diagnosis in this case.

Management

In our case a cesarean section was performed near term. Once the baby was born the diagnosis of teratoma was made with the help of CT and MRI. Surgery was performed successfully and the pathological diagnosis confirmed the impression of hemangioma with part composed of cavernous lymphangioma [29] . These are the photographs pre and postoperative photographs of the neck mass of the newborn.

   

and post op:

 
(sorry but the original images are slightly out of focus!)

Hemangioma

Hemangioma are classified as:

  1. Capillary (strawberry) hemangioma
  2. Cavernous hemangioma
  3. Mixed hemangioma

Strawberry hemangiomas are red protuberant masses that may occur on any area of the body but in particular on the face, scalp, back, and anterior chest. 1-3% of infants have some and girls are 3 times more likely then boys to have some. They may be solitary or multiple. Spontaneous regression is common (60% of these lesions involute with the first 5 year, and most of the rest by 10 years). It has been reported that those diagnosed prenatally migh actually involute faster than those diagnosed postnatally [30] . MRI has occasionally been performed [31] to differentiate from a cephalocele [32] , but the ultrasound appearance is usually characteristic [33] , [34] , [35] , [36] , [37] . These are commonly found in the head [38] , heart [39] , [40] , limbs [41] , [42] and liver [43] , [44] , [45] , abdomen [46] , skin [47] and cord [48] . A rare complication is a thrombocytopenic coagulopathy: the Kasabach-Merritt syndrome.

Cavernous hemangiomas are masses of dilated vessels deep in the skin. They appear as pale, skin-colored, red, or blue masses that are not as sharply defined as the strawberry hemangiomas. They too may undergo spontaneous resolution. Prenatally they may be responsible for hydrops [49] .

Cavernous lymphangioma

Lymphangiomas are congenital malformations of lymphatic vessels. They are the second most common benign vascular tumor in children and are classified in three types:

1)          lymphangioma simplex, which have small lymphatic channels the size of capillary vessels;

2)          cavernous lymphangioma, which are dilated lymphatic channels, with a fibrous capsule; and

3)          cystic hygroma, are lymphatic cysts lined with endothelium found in the neck or axilla but in other location like the thorax inguinal region…

Most of these lesions are present birth, and 90% are recognized before 3 years of age. Small phlebotliths inside the lesion [50] may appear as the bright echoes. They have been found in many areas including the nose [51] , mouth [52] , breast [53] , chest [54] , [55] , [56] , abdomen [57] , retroperitoneum [58] , skin [59] , genitalia [60] and a few prenatal diagnoses have been made33 ,37 ,38 . These slow growing lesions usually infiltrate adjacent tissues and may thus be difficult to extirpate. The local recurrence rate is 6% for incomplete excision in cases of complicated surgery27 . Yet surgical treatment is the best.

References

[1] Chen CP, Chen HC, Liu FF, Jan SW, Lin SP, Sheu JC, Chen BF Progressive fetal axillary cystic lymphangioma with coexistent naevus flammeus. Br J Dermatol 1997 Jan;136(1):102-4

[2] Gimeno Aranguez M, Colomar Palmer P, Gonzalez Mediero I, Ollero Caprani JM The clinical and morphological aspects of childhood lymphangiomas: a review of 145 cases. An Esp Pediatr 1996 Jul;45(1):25-8

[3] Blythe WR, Logan TC, Holmes DK, Drake AF Fibromatosis colli: a common cause of neonatal torticollis. Am Fam Physician 1996 Nov 1;54(6):1965-7

[4] Kimura C, Kitamura T, Sugihara T A case of congenital infantile fibrosarcoma of the right hand. J Dermatol 1998 Nov;25(11):735-41

[5] Pousti TJ, Upton J, Loh M, Grier H Congenital fibrosarcoma of the upper extremity. Plast Reconstr Surg 1998 Sep;102(4):1158-62

[6] Carpenter C, Noonan C, Duane N, Maloney PA, Stebbins K Congenital infantile fibrosarcoma: a case study. Neonatal Netw 1998 Apr;17(3):15-21

[7] Lee MJ, Cairns RA, Munk PL, Poon PY Congenital-infantile fibrosarcoma: magnetic resonance imaging findings. Can Assoc Radiol J 1996 Apr;47(2):121-5

[8] Blocker S, Koenig J, Ternberg J Congenital fibrosarcoma. J Pediatr Surg 1987 Jul;22(7):665-70

[9] Daou RA, Attia EL, Viloria JB Malignant fibrous histiocytomas of the head and neck. J Otolaryngol 1983 Dec;12(6):383-8

[10] Dubois J, Patriquin HB, Garel L, Powell J, Filiatrault D, David M, Grignon A Soft-tissue hemangiomas in infants and children: diagnosis using Doppler sonography. AJR Am J Roentgenol 1998 Jul;171(1):247-52

[11] Yang WT, Ahuja A, Metreweli C Sonographic features of head and neck hemangiomas and vascular malformations: review of 23 patients. J Ultrasound Med 1997 Jan;16(1):39-44

[12] Bosch AM, Hack WW, Ekkelkamp S Congenital hemangiopericytoma: two case reports. Pediatr Surg Int 1998 Mar;13(2-3):211-2

[13] Bailey PV, Weber TR, Tracy TF Jr, O"Connor DM, Sotelo-Avila C Congenital hemangiopericytoma: an unusual vascular neoplasm of infancy. Surgery 1993 Nov;114(5):936-41

[14] Eich GF, Hoeffel JC, Tschappeler H, Gassner I, Willi UV Fibrous tumours in children: imaging features of a heterogeneous group of disorders. Pediatr Radiol 1998 Jul;28(7):500-9

[15] Arda IS, Senocak ME Lipoblastomatosis in a newborn: case report. Pediatr Surg Int 1998 Nov;14(1-2):156

[16] Chang PF, Teng RJ, Tsou Yau KI, Chen CL, Chen CC Lipoblastomatosis in a newborn: case report. Pediatr Surg Int 1997;12(1):71-2

[17] Gomez CS, Calonje E, Ferrar DW, Browse NL, Fletcher CD Lymphangiomatosis of the limbs. Clinicopathologic analysis of a series with a good prognosis. Am J Surg Pathol 1995 Feb;19(2):125-33

[18] Kubota A, Imano M, Yonekura T, Hirooka S, Nose K, Oyanagi H, Nakayama M Infantile myofibromatosis of the triceps detected by prenatal sonography. J Clin Ultrasound 1999 Mar-Apr;27(3):147-50

[19] Johnson GL, Baisden BL, Fishman EK Infantile myofibromatosis. Skeletal Radiol 1997 Oct;26(10):611-4

[20] Chung EB, Enzinger FM Infantile myofibromatosis. Cancer 1981 Oct 15;48(8):1807-18

[21] Chung CJ, Armfield KB, Mukherji SK, Fordham LA, Krause WL Cervical neurofibromas in children with NF-1. Pediatr Radiol 1999 May;29(5):353-6

[22] Kapadia SB, Meis JM, Frisman DM, Ellis GL, Heffner DK Fetal rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study of 24 cases. Hum Pathol 1993 Jul;24(7):754-65

[23] Anderson DR, Narla LD, Dunn NL Subcutaneous fat necrosis of the newborn. Pediatr Radiol 1999 Oct;29(10):794-6

[24] Batsakis JG, el-Naggar AK, Luna MA Teratomas of the head and neck with emphasis on malignancy. Ann Otol Rhinol Laryngol 1995 Jun;104(6):496-500

[25] Coffin CM, Dehner LP Soft tissue tumors in first year of life: a report of 190 cases. Pediatr Pathol 1990;10(4):509-26

[26] Coffin CM, Dehner LP Vascular tumors in children and adolescents: a clinicopathologic study of 228 tumors in 222 patients. Pathol Annu 1993;28 Pt 1:97-120

[27] Gimeno Aranguez M, Colomar Palmer P, Gonzalez Mediero I, Ollero Caprani JM The clinical and morphological aspects of childhood lymphangiomas: a review of 145 cases. An Esp Pediatr 1996 Jul;45(1):25-8

[28] Romero R., Pilu G. Jeanty P., Ghidini A., Hobbins J., Prenatal Diagnosis of Congenital Anomalies pp120-122  Appleton1988

[29] Giacalone PL, Boulot P, Marty M, Deschamps F, Laffargue F, Viala JL Fetal hemangiolymphangioma: a case report. Fetal Diagn Ther 1993 Sep-Oct;8(5):338-40

[30] Boon LM, Enjolras O, Mulliken JB Congenital hemangioma: evidence of accelerated involution. J Pediatr 1996 Mar;128(3):329-35

[31] Kramer LA, Crino JP, Slopis J, Hankins L, Yeakley J Capillary hemangioma of the neck: prenatal MR findings. AJNR Am J Neuroradiol 1997 Sep;18(8):1432-4

[32] Sherer DM, Perillo AM, Abramowicz JS Fetal hemangioma overlying the temporal occipital suture, initially diagnosed by

ultrasonography as an encephalocele. J Ultrasound Med 1993 Nov;12(11):691-3

[33] Suchet IB Ultrasonography of the fetal neck in the second and third trimesters. Part 3. Anomalies of the anterior and anterolateral nuchal region. Can Assoc Radiol J 1995 Dec;46(6):426-33

[34] Shipp TD, Bromley B, Benacerraf B The ultrasonographic appearance and outcome for fetuses with masses distorting the fetal face. J Ultrasound Med 1995 Sep;14(9):673-8

[35] Lasser D, Preis O, Dor N, Tancer ML Antenatal diagnosis of giant cystic cavernous hemangioma by Doppler velocimetry. Obstet Gynecol 1988 Sep;72(3 Pt 2):476-7

[36] McGahan JP, Schneider JM Fetal neck hemangioendothelioma with secondary hydrops fetalis: sonographic diagnosis. J Clin Ultrasound 1986 Jun;14(5):384-8

[37] Grundy H, Glasmann A, Burlbaw J, Walton S, Dannar C, Doan LHemangioma presenting as a cystic mass in the fetal neck. J Ultrasound Med 1985 Mar;4(3):147-50

[38] Boulot P, Deschamps F, Montoya F, Montoya P, Couture A, Ferran JL, Lefort G Prenatal aspects of giant fetal cranial haemangio-endothelioma. Prenat Diagn 1996 Apr;16(4):357-9

[39] Eckstein FS, Heinemann MK, Mielke GJ, Greschniok A, Bader P, Ziemer G Resection of a large right atrial hemangioma in a neonate after prenatal

diagnosis. Ann Thorac Surg 1999 Sep;68(3):1074-5

[40] Tseng JJ, Chou MM, Lee YH, Ho ES In utero diagnosis of cardiac hemangioma. Ultrasound Obstet Gynecol 1999 May;13(5):363-5

[41] Raman S, Ramanujam T, Lim CT Prenatal diagnosis of an extensive haemangioma of the fetal leg: a case report. J Obstet Gynaecol Res 1996 Aug;22(4):375-8

[42] Goncalves LF, Pereira ET, Parente LM, Vitorello DA, Barbosa UC, Saab Neto JA Cutaneous hemangioma of the thigh: prenatal diagnosis. Ultrasound Obstet Gynecol 1997 Feb;9(2):128-30

[43] Morris J, Abbott J, Burrows P, Levine D Antenatal diagnosis of fetal hepatic hemangioma treated with maternal

corticosteroids.Obstet Gynecol 1999 Nov;94(5 Pt 2):813-5

[44] Chuileannain FN, Rowlands S, Sampson A Ultrasonographic appearances of fetal hepatic hemangioma. J Ultrasound Med 1999 May;18(5):379-81

[45] Dreyfus M, Baldauf JJ, Dadoun K, Becmeur F, Berrut F, Ritter J Prenatal diagnosis of hepatic hemangioma. Fetal Diagn Ther 1996 Jan-Feb;11(1):57-60

[46] Maynor CH, Hertzberg BS, Kliewer MA, Heyneman LE, Carroll BA Antenatal ultrasonographic diagnosis of abdominal wall hemangioma: potential to

simulate ventral abdominal wall defects. J Ultrasound Med 1995 Apr;14(4):317-9

[47] Treadwell MC, Sepulveda W, LeBlanc LL, Romero R Prenatal diagnosis of fetal cutaneous hemangioma: case report and review of the

literature. J Ultrasound Med 1993 Nov;12(11):683-7

[48] Miller KA, Gauderer MW Hemangioma of the umbilical cord mimicking an omphalocele. J Pediatr Surg 1997 Jun;32(6):810-2

[49] Sharara FI, Khoury AN Prenatal diagnosis of a giant cavernous hemangioma in association with nonimmune

hydrops. A case report. J Reprod Med 1994 Jul;39(7):547-9

[50] Murphey MD, Fairbairn KJ, Parman LM, Baxter KG, Parsa MB, Smith WS From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics 1995 Jul;15(4):893-917

[51] Hobby JL, Tiernan E, Mayou BJ The "Pinocchio" nasal deformity due to cavernous lymphangioma. J R Soc Med 1995 Sep;88(9):535P-536P

[52] Paladini D, Morra T, Guida F, Lamberti A, Martinelli P Prenatal diagnosis and perinatal management of a lingual lymphangioma. Ultrasound Obstet Gynecol 1998 Feb;11(2):141-3

[53] Chiba T, Ibrahim M Cavernous lymphangioma of the breast: case report of an infant. Nippon Geka Hokan 1995 Jan 1;64(1):23-6

[54] Lehka J, Pinter L Cavernous lymphangioma. Rozhl Chir 1996 Oct;75(10):474-6

[55] Yu CH, Lu WT, Wu FF, Liao YJ, Liang WW Mediastinal lymphangioma in an infant. J Formos Med Assoc 1996 Mar;95(3):255-7

[56] Matsui H, Andou S, Satake A, Sakakibara K, Uragami T, Kobayashi T Cavernous lymphangioma of mediastinum--a case report. Nippon Kyobu Geka Gakkai Zasshi 1993 Jul;41(7):1257-61

[57] Mentzel HJ, Schramm D, Vogt S, Reuter A, Mentzel T, Kaiser WA Intra-abdominal lymphangioma in a newborn. J Clin Ultrasound 1998 Jul-Aug;26(6):320-2

[58] Malnofski MJ, Poulton TB, Nazinitsky KJ, Hissong SL Prenatal ultrasonic diagnosis of retroperitoneal cystic lymphangioma. J Ultrasound Med 1993 Jul;12(7):427-9

[59] Gollin YG, Siddiqui SY Midtrimester diagnosis of a diffuse cutaneous cavernous lymphangioma. Obstet Gynecol 1998 May;91(5 Pt 2):861

[60] Allen-Davis JT, Russ P, Karrer FM, Shroyer K, Ruyle S, Odom LF Cavernous lymphangioma presenting as a vaginal discharge in a six-year-old female: a case report. J Pediatr Adolesc Gynecol 1996 Feb;9(1):31-4

Back to case
Help Support TheFetus.net :