횡문근육종 환아의 혈청 Neutral Ribonuclease의 분리와 성상에 관한 연구

박성원,이상훈,한중수,고재경 한양대학교 의과대학 1992 한양의대 학술지 Vol.12 No.2

The activity of neutral ribonuclease (RNase) was determined in serum of patients with rhabdomyosarcoma to find out whether the serum enzyme could be used as a marker for rhabdomyosarcoma. Also analysed were isolation patterns of proteins and neutral RNases to investigate the persence of RNase specific to the rhabdomyosarcoma. In serum of patients with rhabdomyosarcoma, activities of amylase, alkaline phosphatase and 5'-nucleotidase were unchanged, but neutral RNase as a marker for the myosarcoma. Neutral RNases in serum of the rhabdomyosarcoma and in its control serum were separated by a DEAE-cellulose column chromatography into 5peaks each and the peak specific to the myosarcoma was not found. The peak I neutral RNase isozyme was, however, observed to be greater in rhabdomyosarcome serum than in the control serum and the peak Ⅴ neutral RNase isozyme was lesser in the myosarcoma than in the control. The peak I neutral RNase isozyme isolated from the rhabdomyosarcoma serum was active toward poly C and less active toward poly U and RNA, indicating the enzyme was the secretory type of RNase in nature. No activity was observed toward purine poly ribonucleotides, polyeosyribonucleotides and double stranded polyribonucleotides. Of the geteropolyribonucleotides used as substrate in the present study, the peak I neutral RNase isozyme from rhabdomyosarcoma serum was highly active toward poly AC and active toward poly ACU, AU, CI, CIU in the decreasing order. No or little activity was observed with poly AG, AGU and ACG. This would indicate that the RNase isozyme is active towasrd linkages made of cytosine and inactive toward linkages with guanine. Studies on the substrate specificity showed differences between the peak I neutral RNase isozymes from rhabdomyosarcoma and control serum, exhibiting decrease in the ratio of poly U/ poly C, RNA/ poly C and heteropolyribonucleotides/ poly C activities in the myosarcoma. This suggerts that the peak I neutral RNase isozyme minght be specific to the rhabdomyosarcoma. DEAE peak I proteins isolated from rhabomyosarcoma and control serum were further separated by a HPLC into 3 peaks each, the protein pattern was similar in DEAE peak I of both serum. The results obtained in the present study indicate that 1) neutral RNaes activity in serum of rhabdomyosarcoma was significantly increased suggesting the possible use of the RNase as a marker ofr the rhadbomyosarcoma and that 2) the peak I neutral RNase as a marker for the rhadbomyosarcoma and that cific to the myosarcome, exhibitin the difference of substrate specifity between the rhabdomyosarcoma and control.

[P304] Primary cutaneous embryonal type rhabdomyosarcoma

( Jae Won Lee ),( Sang Kyum Kim ),( Chang Ook Park ),( Kee Yang Chung ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Primary cutaneous presentation of rhabdomyosarcoma accounts for less than 1% of rhabdomyosarcoma. Thus the primary cutaneous rhabdomyosarcoma of embryonal type is a very rare entity with only 10 cases reported in published work. A 5-year-old female was referred to our department for a single nodule on her face. An 8x8mm-sized, flesh-colored protruded nodule, which was located on her left cheek, first appeared 3 years ago and had increased gradually in size. Histopathologic examination showed alternating myxoid and cellular zones composed of ovoid cells with hyperchromatic nuclei and indistinct cytoplasm. Immunohistochemical staining showed positivity for desmin, myogenin and CD56. There was no evidence of involvement of other organs in various examinations including bone marrow biopsy and PET-CT. The lesion was diagnosed as embryonal primary cutaneous rhabdomyosarcoma and the patient underwent Mohs surgery followed by rhabdomyosarcoma-specific chemotherapy. Given the paucity of experience with primary cutaneous presentation of these tumors, there may be difficulties in the differential diagnosis of sarcomatoid tumors with histopathology showing small round blue cells. In this case, positivity for desmin and myogenin with negativity for several other markers in immunohistochemistry facilitated the diagnosis. To the best of our knowledge, this is the first case of embryonal primary cutaneous rhabdomyosarcoma reported in Korean patients.

인두 횡문근육종 1예

장은석,이주상,서진원,박범정 대한이비인후과학회 2010 대한이비인후과학회지 두경부외과학 Vol.53 No.11

Rhabdomyosarcoma is a highly aggressive malignant tumor with an incidence of 1/500,000 children per year. It originates from mesenchymal cell to invade soft tissue, developing into a highly aggressive malignant tumor. Rhabdomyosarcoma consists of the following four pathologic groups-embryonal, botryoid, alveolar, and pleomorphic type. While head and neck is the most common site of the embryonal rhabdomyosarcoma, the pharynx is rarely reported as a primary site of rhabdomyosarcoma. Recently, the 5-year survival rate of rhabdomyosarcoma has been greatly increased by combining therapy with radical surgery, although it still has poor prognosis. We present a case of rhabdomyosarcoma in the pharynx. Korean J Otorhinolaryngol-Head Neck Surg 2010;53:719-22

68세 여자의 눈물주머니에 생긴 원발성 횡문근육종

임지원,이선주,Ji Won Lim,Sun Joo Lee 대한안과학회 2006 대한안과학회지 Vol.47 No.9

Purpose: Rhabdomyosarcoma of the lacrimal sac is extremely rare. There has only been one case in this country, during the 30s, and to our knowledge, there are no reports of primary rhabdomyosarcoma on the lacrimal sac. We report a case of primary rhabdomyosarcoma of the lacrimal sac in a 68-year-old woman Methods: A 68-year-old female patient presented with tearing of the right eye. A lacrimal sac mass extending to the proximal nasolacrimal duct was detected during the dacryocystorhinostomy, and incisional biopsy was performed. Histopathology revealed a rhabdomyosarcoma of the right lacrimal sac. After systemic chemotherapy, complete excision of the tumor through the medial maxilla was performed. Results: The pathologic diagnosis of the excised mass was embryonal type rhabdomyosarcoma. There was no evidence of tumor recurrence in the lacrimal sac or paranasal sinus during 9 months of follow-up. Conclusions: Although rhabdomyosarcoma is very uncommon, this case provides the rationale for including it in differential diagnosis of a mass in lacrimal sac.

자궁경부에 발생한 배아성 횡문근육종 2예

박정열,조준식,김대연,이동헌,김종혁,김용만,김영탁,목정은,남주현 대한부인종양 콜포스코피학회 2002 Journal of Gynecologic Oncology Vol.13 No.3

횡문근육종은 소아와 청소년기에 가장 흔한 연부조직 육종이다. 발생 부위는 일반적으로 두부 및 경부, 그 다음으로 비뇨생식계이다 조직학적으로 배아성 횡문근육종, 국화상 육종, 폐포성 횡문근육종, 다형성 횡문근육종으로 나누어진다. 횡문근육종의 약 20%가 비뇨생식기에서 발생하고, 50% 이상이 배아성 횡문근육종이다. 여성 생식기의 배아성 횡문근육종은 드문 악성 종양으로,주로 유소아의 질에서 발생하며, 자궁경부에서 발생하는 횡문근육종은 청소년기에 흔하고, 질에서 기원하는 횡문근육종이 자궁경부에 기원하는 경우보다 5배 정도 많다. 폐경기여성에서는 자궁체부에서 가장 흔히 발생한다. 비뇨생식기의 횡문근육종의 치료는 과거에는 골반장기 적출술만으로 치료를 시도하였으나. 서서히 다중 항암화학요법, 방사선요법, 근치적 절제술을 포함한 병합요법으로 바뀌어가고 있으며, 이에 따라 생존율이 현저하게 향상되었다. 이에 저자들은 최근 본원 산부인과에서 자궁경부에 발생한 배아성 횡문근육종 2예를 경험하였기에 문헌 고찰과 아울러 보고하는 바이다. Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and young adult. Genitourinary tract is the econd most common site of rhabdomyosarcoma. Rhabdomyosarcoma can be classified into one of four major types; embryonal, alveolar, pleomorphic and undifferentiated. Embryonal rhabdomyosarcoma of the female genital tract is rare malignant tumor and usually occurs during childhood in the vagina. In rare cases, rhabdomyosarcoma can originate in the uterine cervix, with a peak incidence in the second decade. Recently we have experienced two cases of embryonal rhabdomyosarcoma of the uterine cervix. These cases are presented with a brief review of the literature.

FCP 6 : Rhabdomyosarcoma mistaken for cellulitis occurred on the face

( Yeon A No ),( In Kwon Yeo ),( Seong Jun Seo ),( Chang Kwun Hong ) 대한피부과학회 2014 대한피부과학회 학술발표대회집 Vol.66 No.1

Rhabdomyosarcoma is a rare malignant soft tissue neoplasm with skeletal muscle differentiation. Cutaneous rhabdomyosarcoma is exceedingly rare, and in most cases, it represents metastatic disease or the dermal involvement by a tumor arising on the underlying soft tissues. A 49-year-old women complained with unilateral erythematous swollen and sclerosing patch on the right face for 1 month. She had a past history of botulinum toxin injection therapy on the both massater muscle and periorbital area 3 months ago. In radiologic findings, myositis and cellulitis were diagnosed. As she didn’t show any improvement for treatment of myositis and cellulitis, skin biopsy was performed. Histopathologic examination was compatible with rhabdomyosarcoma. Herein we present a case of rhabdomyosarcoma mistaken for cellulitis on the face.

부인두강에 발생한 횡문근육종 1예

박의현,이기정,박민우,정광윤 대한이비인후과학회 2014 대한이비인후과학회지 두경부외과학 Vol.57 No.11

Rhabdomyosarcoma is a highly aggressive malignant tumor that originates from mesenchymal cell and invades soft tissue. While the head and neck is the common site of the rhabdomyosarcoma, the parapharyngeal space is rarely reported as a primary site of rhabdomyosarcoma. Therefore, we present a case of rhabdomyosarcoma in the parapharyngeal space. Korean J Otorhinolaryngol-Head Neck Surg 2014;57(11):774-7

하악에 발생한 횡문근육종

윤숙자,강병철 대한구강악안면방사선학회 2004 Imaging Science in Dentistry Vol.34 No.2

Rhabdomyosarcoma, when it occurs in the head and neck, is primarily found in children. Alveolar rhabdomyosarcoma is rarely seen in the oral lesion, comparing to the embryonal and the pleomorphic variants. This is a report of a case of alveolar rhabdomyosarcoma in the mandible in a ten-year old girl who complained of a non-painful swelling on the right cheek. The right lower 1st molar was mobile. Her radiographs revealed an extensive radiolucency with somewhat irregular border on the right mandibular ramus. The right mandibular 1st and 2nd molars lost their lamina dura and were floating. CT images revealed smooth-outlined soft tissue mass occupying the pterygomandibular space, the infratemporal space, and the masseteric muscle with thinning and perforation of the right mandibular angle and ramus. Histopathological and immunohistochemical findings established the final diagnosis of alveolar rhabdomyosarcoma.

성인에서 발생한 비강내 배아성 횡문근 육종 1예

이영택,김광일,이창기,임영환 대한이비인후과학회 2000 대한이비인후과학회지 두경부외과학 Vol.43 No.12

Clinical-pathologic profile of head and neck rhabdomyosarcoma in children: a systematic review

Ludimila Lem es Moura(Ludimila Lemes Moura ),Beatriz Della Terra Mouco Garrido(Beatriz Della Terra Mouco Garrido ),Nelson Leonel Del Hierro Polanco(Nelson Leonel Del Hierro Polanco ),Mattheus Augusto 대한구강악안면외과학회 2023 대한구강악안면외과학회지 Vol.49 No.2

This systematic review aimed to analyze the clinicopathological profile and relevant prognostic factors of head and neck rhabdomyosarcoma in pediatric patients. The search was carried out in the electronic search portals PubMed, Lilacs, Embase, Scopus, and Web of Science. The search yielded studies that were then analyzed regarding study topic, data extraction, and risk of bias using the STROBE (Strengthening the Reporting of Observational Studies) guidelines. Finally, three studies were included for qualitative analysis. Most of the cases involved embryonic and alveolar rhabdomyosarcoma. Expression of MYOD1 was highly correlated with diagnosis of spindle cell/sclerosing rhabdomyosarcoma, which appears to have a poor prognosis in children. Furthermore, tumor size <5 cm and absence of metastasis accompanied by complete resection and administration of adjuvant therapies such as chemotherapy and radiotherapy favored a better prognosis.