Neonatal giant forehead Hemangiopericytoma: A rare Occurrenc
A 3-day-old girl was delivered normally with a giant tumor localized mainly in the right frontal region. On physical examination, her head circumference was 39 cm. Additionally, a 9 × 7.1 × 5.1 cm soft, rounded and well-defined mass with a broad base occupied most of the forehead. The surface of the mass was filled with vascularity (Fig. 1A), and other clinical examinations showed that the mass had an intact status. A diagnosis of hemangioma was made at admission.

A CT scan revealed a large mass tumor located in the front of the head, and computed tomography angiography (CTA) demonstrated that the blood supply to the vascularized tumor originated mainly from the bilateral supratrochlear artery and partly from the distal part of the right superficial temporal artery (Fig. 2A). Moreover, MRI showed a right extracranial mass with heterogeneous intensity on contrast-enhanced CT (Fig. 2B) and isointensity on T1-weighted (Fig. 2C) and T2-weighted images (Fig. 2D).

After a thorough preoperative evaluation with a clinical collaborative multidisciplinary team a surgery was performed. During the operation, the tumor was completely removed successfully, and the eroded frontal bone was abraded with grinding drill of milling cutter. Following resection, the skin of scalp was successfully reconstructed.

After the operation, the infant was sent to the intensive care unit to monitor her vital signs. With careful wound nursing, she was discharged in good condition four weeks later. She did not receive chemotherapy or radiotherapy. No evidence of recurrence or metastasis was found during follow-ups performed 6 months after. After a 5-year follow-up period, the wound could not be found without careful examination (Fig. 1B), and there were no signs of recurrence or metastasis of the neoplasm on a CT scan (Fig. 2E).

Learning points:-
1. Hemangiopericytoma (HPC) includes two distinct clinical types: infantile (occurring at less than 1 year old) and adult (occurring at more than 1 year old). Infantile HPC can occur anywhere in the body, including primary soft tissues, deeper structures and the lower extremities, but the most common site is the head and neck.
2. The clinical symptoms of infantile HPC can include hemorrhage, compression of local structures, and even sudden death.
3. It represent a diversity of neoplasms ranging from well- to poorly differentiated tumors (low- to high-grade, respectively).
4. They are divided into three grades based on histological subtype, including
a) WHO grade I (SFT);
b) WHO grade II (HPC), which contains more pump cells and
has a “staghorn” vasculature; &
c) WHO grade III (anaplastic HPC), which shows more than 5
mitoses per high-power field (HPF).
5. According to the literature, the mainstay treatment for HPC is total tumor resection whenever possible. The risk of surgery-related morbidity and mortality in infantile HPCs increases with the amount of bleeding, and this may be the greatest barrier to complete resection.

Overall, the outcomes of infantile HPC are different from those found in adult HPC due to differences in treatments and tumor locations.

Source: https://journals.lww.com/md-journal/Fulltext/2019/11220/Case_report__neonatal_giant_forehead.14.aspx
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Dr. J●●●●h S●●●y
Dr. J●●●●h S●●●y Dentistry
Great surgery
Nov 23, 2019Like
Dr. A●●●y P●●●●●h
Dr. A●●●y P●●●●●h Pathology
Rare
Nov 25, 2019Like
K●●●●●n Y●●i
K●●●●●n Y●●i General Medicine
Very nice
Dec 5, 2019Like