1
原文題目(出處)：
Lipomatosis of the neck: Case report and literature review. Int J Pediatr Otorhinolaryngol Extra
2010;5:39-41
原文作者姓名：
Sreekrishna Kanth Donepudi
a, James C. Greene
b, Rose Mary S. Stocks
a,*
通訊作者學校： a Department of Otolaryngology-Head and Neck Surgery, University of Tennessee Health Science Center, 910 Madison Ave, Room 428, Memphis, TN 38163, United States
b University of Tennessee Health Science Center, 910 Madison Ave, Room 428, Memphis, TN 38163, United States
報告者姓名(組別)： 李威誼(Intern L組)
報告日期： 2010.07.12
內文：
Introduction
1. lipoma
a. 脂肪瘤是由於成熟的皮下脂肪組織異常增生而引起的良性腫瘤。
b. 成人中最常見的 mesenchymal lesion。大約有13%的lipoma發生在頭頸部，而 整體約有5%的復發率。
c. lipoma lesion通常是分散且encapsulated。
d. 組織學上，lipomatosis 和lipoma 最大的不同就是lipomatosis缺乏 circumscription
2. lipomatosis
a. lipomatosis是過度增生、poorly circumscribed和diffuse的成熟脂肪細胞 b. 典型的Lipomatosis一般不是neoplastic就是hamartomatous。
c. 它通常會和多種組織或結構發育異常同時存在，而其他這些異常疾病的特性 常會掩蓋了lipomatosis，也因此造成在診斷上的困難。
3. 頭頸部的lipomatosis的differential diagnosis : a. steroid-induced mediastinal lipomatosis
b. congenital infiltrating lipomatosis of the face c. lipoblastomatosis
d. diffuse lipomatosis
e. Bannayan- Zonana syndrome
f. encephalocraniocutaneous lipomatosis
Nguyen et al提出的paper中有提到 Lipomas and lipomatosis with mediastinal involvment。本篇只就第二個 idiopathic mediastinal lipomatosis 的case做探討。
Case report
1. 病人是一個 2.5歲的小女孩，被發現在右邊鎖骨之上有一個無痛的mass約六個 月，關於呼吸或吞嚥困難方面並沒有相關的症狀紀錄。
2. Past medical history: asthma and recurrent otitis media.
3. Birth history: unremarkable 4. PE(physical exam):
neck mass size:2 cm ＊3 cm soft of palpation
transilluminated upon examination with a light source.
5. 相關的頭頸部檢查是正常的
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6. 從Computerized tomography (CT) 可發現到mass中fat density 從cricoid cartilage 後方mediastinun旁邊一直延伸到 trachea和esophagus。 mass的組織外並沒有 envelope包覆
mediastinal lipomatosis presenting as a neck mass in the pediatric population.
2. Case report
A 2.5-year-old female presented with a painless right supraclavicular mass of less than 6 months duration.
No associated symptoms of airway compromise or swallowing difficulty were reported. Past medical history was remarkable for asthma and recurrent otitis media. Birth history was unremarkable. On physical exam, the neck mass was 2 cm ! 3 cm in size, soft to palpation, and transilluminated upon examination with a light source. The remainder of the head and neck examination was normal. Compu- terized tomography (CT) revealed a mass of fat density extending from the level of the cricoid car- tilage inferiorly into the mediastinum adjacent to the trachea and esophagus, without enveloping these structures (see Fig. 1). There was no evidence of invasion or bony destruction.
Incisional biopsy was taken in the operating room using a transverse incision anterior to the sterno- cleidomastoid muscle border. A fatty mass was noted without evidence of fluid or unusual vascu- larity. The biopsy was taken and the wound was closed in layers. Histologic evaluation was consis- tent with lipomatosis (seeFig. 2).
Given the mediastinal extent of the tumor, sur- gical resection would have included thoracotomy or sternotomy with associated morbidity. For this rea- son, nonoperative management was recommended and agreed upon with the family. After 2 years, the child remained free of symptoms, and yearly ima- ging showed no enlargement of the mass.
3. Discussion
A review of the English literature for lipomatosis returned a surprisingly diverse subset of rare dis- eases. Multiple symmetrical lipomatosis (or Made- lung’s disease) was first described by Brodie in 1846 and is perhaps one of the more well-known causes of lipomatosis of the neck. This disorder is char- acterized by diffuse symmetric deposition of fat in the neck, face, shoulder, upper extremities, and trunk[5].
Lipomatosis secondary to extended corticoster- oid use or Cushing’s syndrome is also commonly reported. These lesions can manifest in the med- iastinum, mimicking cardiomegaly on imaging with widening of the mediastinal silhouette [6]. This lesion can be seen in a child taking long-term ster- oids for asthma or graft versus host disease after bone marrow transplant[3].
Congenital infiltrating lipomatosis of the face is a rare disorder that presents at birth on the cheek. It has ill-defined borders and infiltrates muscle and soft tissue, making excision difficult and recurrence likely. There may be hypertrophy of underlying bone. Definitive excision and reconstruction are delayed until adolescence to allow regression of the buccal fat pad, minimize the risk of damage to the facial nerve, and to have a mature contral- ateral cheek contour to match[2].
Lipoblastomas are benign tumors of embryonic adipose tissue that occur in infancy and early child- hood, with 90% occurring before 3 years of age[3].
They represent 2% of all pediatric soft tissue tumors [7]. While typically presenting as a rapidly growing soft tissue mass involving the extremities, these lesions have been reported in the head, neck, and mediastinum [3]. Lipoblastomatosis, as expected, can be defined as a locally invasive lipoblastoma.
Sun et al.[7] reported that only 10 cases of lipo- blastomatous tumors have been reported.
40 S.K. Donepudi et al.
Fig. 1 CT scan of mediastinum showing mass (arrow) adjacent to trachea and esophagus without enveloping them.
Fig. 2 Low magnification view of adipocytes with focally increased fibroconnective tissue.
7. 並沒有 evidence顯示有invasion或bony destruction.
8. 開刀取Incisional biopsy，使用transverse incision從sternocleidomastoid muscle border切. 這個fatty mass並沒有含有fluid或unusual vascularity的跡象。
9. Histologic 的評估認為是lipomatosis (see Fig. 2).
mediastinal lipomatosis presenting as a neck mass in the pediatric population.
2. Case report
A 2.5-year-old female presented with a painless right supraclavicular mass of less than 6 months duration.
No associated symptoms of airway compromise or swallowing difficulty were reported. Past medical history was remarkable for asthma and recurrent otitis media. Birth history was unremarkable. On physical exam, the neck mass was 2 cm ! 3 cm in size, soft to palpation, and transilluminated upon examination with a light source. The remainder of the head and neck examination was normal. Compu- terized tomography (CT) revealed a mass of fat density extending from the level of the cricoid car- tilage inferiorly into the mediastinum adjacent to the trachea and esophagus, without enveloping these structures (see Fig. 1). There was no evidence of invasion or bony destruction.
Incisional biopsy was taken in the operating room using a transverse incision anterior to the sterno- cleidomastoid muscle border. A fatty mass was noted without evidence of fluid or unusual vascu- larity. The biopsy was taken and the wound was closed in layers. Histologic evaluation was consis- tent with lipomatosis (seeFig. 2).
Given the mediastinal extent of the tumor, sur- gical resection would have included thoracotomy or sternotomy with associated morbidity. For this rea- son, nonoperative management was recommended and agreed upon with the family. After 2 years, the child remained free of symptoms, and yearly ima- ging showed no enlargement of the mass.
3. Discussion
A review of the English literature for lipomatosis returned a surprisingly diverse subset of rare dis- eases. Multiple symmetrical lipomatosis (or Made- lung’s disease) was first described by Brodie in 1846 and is perhaps one of the more well-known causes of lipomatosis of the neck. This disorder is char- acterized by diffuse symmetric deposition of fat in the neck, face, shoulder, upper extremities, and trunk[5].
Lipomatosis secondary to extended corticoster- oid use or Cushing’s syndrome is also commonly reported. These lesions can manifest in the med- iastinum, mimicking cardiomegaly on imaging with widening of the mediastinal silhouette [6]. This lesion can be seen in a child taking long-term ster- oids for asthma or graft versus host disease after bone marrow transplant[3].
Congenital infiltrating lipomatosis of the face is a rare disorder that presents at birth on the cheek. It has ill-defined borders and infiltrates muscle and soft tissue, making excision difficult and recurrence likely. There may be hypertrophy of underlying bone. Definitive excision and reconstruction are delayed until adolescence to allow regression of the buccal fat pad, minimize the risk of damage to the facial nerve, and to have a mature contral- ateral cheek contour to match[2].
Lipoblastomas are benign tumors of embryonic adipose tissue that occur in infancy and early child- hood, with 90% occurring before 3 years of age[3].
They represent 2% of all pediatric soft tissue tumors [7]. While typically presenting as a rapidly growing soft tissue mass involving the extremities, these lesions have been reported in the head, neck, and mediastinum [3]. Lipoblastomatosis, as expected, can be defined as a locally invasive lipoblastoma.
Sun et al. [7]reported that only 10 cases of lipo- blastomatous tumors have been reported.
40 S.K. Donepudi et al.
Fig. 1 CT scan of mediastinum showing mass (arrow) adjacent to trachea and esophagus without enveloping them.
Fig. 2 Low magnification view of adipocytes with focally increased fibroconnective tissue.
10. 在處理有mediastinal extent的tumor，一般會採取的surgical resection有
thoracotomy或sternotomy，但考慮此病患的狀況及存活率的問題 ，因此在家 屬的同意下決定不採取手術。
11. 兩年後，p’t仍然沒有臨床症狀，mass也沒有擴大的跡象。
Discussion
1. review關於lipomatosis的English literature發現其與多種罕見疾病有關連。
2. Multiple symmetrical lipomatosis (or Made- lung’s disease) 在1846年由Brodie首 次提出，而這也可能是造成頸部lipomatosis最為人知的的原因之一 。他的特 色是diffuse symmetric deposition of fat in the neck, face, shoulder, upper
extremities, and trunk
3. 有報告指出Lipomatosis 和corticosteroid use或Cushing’s syndrome也有關連。
lesions會明顯出現在mediastinum，類似心臟擴大的影像伴隨有橫膈變寬的影 像. 這種lesion會出現在長期因asthma或 bone marrow transplant做骨髓移植而使 用steroid的小孩。
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4. 罕見的臉部Congenital infiltrating lipomatosis會出現在新生兒的臉頰。他的邊 界不明顯並且會滲透到肌肉和軟組織，不容易excision且容易復發。它也可能 會使下方的bone hypertrophy。因此若要做Definitive excision and reconstruction 一般會等到青少年時期，讓mass regression 到buccal fat pad，降低傷害到facial nerve的風險並讓mature contralateral cheek contour可以 match。
5. Lipoblastomas 是embryonic adipose tissue的良性tumor，會發生在嬰兒和年紀 較小的兒童，有90% 會發生在3歲之前。lipoblastomas約佔全部pediatric soft tissue tumors的2%。 典型的lipoblastoma是生長快速的soft tissue mass 會involve 到四肢，lesion會出現在head, neck, and mediastinum [3].而 Lipoblastomatosis則 是一種 locally invasive lipoblastoma. 根據Sun et al.的報告指出目前只有10例 lipoblastomatous tumors被發表過。
6. 許多的罕見兒童頭頸部疾病都會伴隨有lipomatosis。
7. Diffuse lipomatosis是一種快速生長擴張的mass，會長在 trunk 或extremity，且 常和tuberous sclerosis、hyperostosis和gigantism有關連。
8. Bannayan-Zonana syndrome會在軀幹或四肢產生lipomatosis、lymphangioma和 hemangiomas ，Macroencephaly和other malformations也可能同時存在。
9. encephalocraniocutaneous lipomatosis會包含有epilepsy、mental retardation和 cerebral malformations及scalp和neck的lipomatosis。
10.lipomatosis的診斷可以靠著病史、PE和影像學檢查
11.congenital malformations的病史、steroid的過度使用、酒精濫用、Cushing’s syndrome或其他endocrinologic abnormalities等等都有可能是造成lipomatosis的 原因。
12.理學檢查可發現lipomatous tissue是柔軟且可動的。當用微光去照射檢查時，
本案例中的p’t的lesion是典型transilluminates。computed tomography (CT)影像 下成熟的脂肪組織呈現low attenuation。lipomatous tissue呈現high intensity在 T1-weighted and T2-weighted magnetic resonance imaging (MRI)
13.只有biopsy和histopathologic examination可以明確的鑑別出lipomatosis和其他 像是lipoblastomatosis和liposarcoma。
14.只要沒有lobule formation和embryonic fat就可以排除lipoblastomatosis。
15.不像lipomas，組織學檢查可發現liposarcomas有lipoblastic的增生、
pleomorphism、mitosis[2].
16.Prognosis和treatment approach因case狀況不同差異很大。
17.infiltrating type of lipomatosis很靠近主要血管或spinal cord往往會有較差的預 後、手術上的困難度也會提高。
18.在本case中，影像中顯示了large lesion擴及到了mediastinum。雖然病人並沒 有症狀顯示有影響到呼吸或其它vital structures而且組織檢查上也排除了惡性 的可能。但治療上還是趨向保守，如果採取積極的手術治療像是thoracotomy 或sternotomy可能會影響到病人的存活率 。所以採取定期追蹤來掌握lesion的 變化。
19.究竟是什麼原因造成這個有asthma and recurrent otitis media病史的病人產生 lipomatosis仍然不明。
20.Pungavkar et al. 曾發表過一個特有的mediastinal lipomatosis的case的病人沒有 相關的已知的risk factor，和本案例類似。本案例中的病人並沒有先天性異常 的病史，家族中也沒有人罹患過lipomatous abnormalities。 也沒有長期使用 steroid、alcohol濫用或 Cushing’s syndrome。
題號 題目題目
1 下列關於lipoma何者正確？下列關於lipoma何者正確？
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(A) lipoma是全身最常見的benign tumor (B) 口腔內的好發位置是soft palate (C) lipoma通常是poor circumscribed (D) 切除後復發率高
答案
( A) 出處：Oral & Maxillofacial Pathology 3rd ed. P.523 P524 出處：Oral & Maxillofacial Pathology 3rd ed. P.523 P524
題號 題目題目
2 下列何者是oral cavity中最常見的liposacroma？下列何者是oral cavity中最常見的liposacroma？
(A) myxoid liposacroma (B) round cell liposaroma
(C) well-deferentiated liposacroma (D) pleomorphic liposaroma
答案
( C) 出處：Oral & Maxillofacial Pathology 3rd ed. P.554 出處：Oral & Maxillofacial Pathology 3rd ed. P.554
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