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.

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