otolaryngologia polska 68 (2014) 42–45
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/otpol
Case report/Kazuistyka
Leiomyosarcoma of the external ear – Case report Mie˛sak gładkokomo´ rkowy ucha – opis przypadku Maciej Rysz 1,*, Janusz Jaworowski 1, Romuald Krajewski 1, Joanna Baran´ ska 2 1
Head and Neck Cancer Department, Surgery Division, Memorial Cancer Center, Head: A. Kawecki MD, PhD, Poland 2 Department of Pathology, Memorial Cancer Center, Warsaw, Head: K. Ptaszyn´ ski MD, Poland
article info
abstract
Article history:
In head and neck region leiomyosarcoma is a very rare finding. This tumor is usually located
Received: 29.01.2012
in abdominal cavity. We report a case of 78-year-old man with tumor of the auricle that was
Accepted: 13.06.2012
resected with 0.7 cm margin. Histological and immunohistochemical analysis revealed the
Available online: 18.06.2012
leiomyosarcoma. Adjuvant therapy was not advised. During 3 years of follow-up there are no signs of recurrence. Review of the literature shows that the survival of patients with leiomyosarcoma in head and neck region seems to be similar to other kinds of sarcomas in
Keywords: Leiomiosarcoma
this localization. It is not clear, how large surgical margins should be to achieve satisfactory
Sarcomas of the head
local control. © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by
Słowa kluczowe: mie˛sak gładkokomo´rkowy mie˛saki głowy
Elsevier Urban & Partner Sp. z o.o. All rights reserved.
Introduction
Case report
Leiomyosarcoma (LMS) is a malignant mesenchymal neoplasm of smooth muscles origin [1]. Two types of LMS – superficial and cutaneous are distinguished. Cutaneous leiomyosarcoma grows slowly, and has better prognosis than subcutaneous leiomyosarcoma [1, 2]. LMS is diagnosed in approximately 6% of all soft tissues sarcomas, and head and neck region sarcomas constitute less than 1% of tumors in this region [1, 3]. Most frequently, LMS is found in uterus, gastrointestinal tract and retroperitoneal space. Location in head and neck region is very rare [4]. We report an extremely rare case of LMS of external ear.
The patient is a 78-year-old man with rapidly growing tumor on the left auricle. A small spot, less than 1 cm in diameter on the ear skin has been observed since about 5 years. Two months before admission the tumor started to grow rapidly. The examination revealed an exophytic tumor on the left auricle measuring 1 1.5 1.5 cm. It was attached to the auricle cartilage, but well separated from the surrounding skin. One centimeter skin margin around the tumor was reddened, painless, without palpable infiltration. There was no evidence of cervical node or distant metastases (T1, N0, M0).
* Corresponding author: Maciej Rysz, Head and Neck Department, Cancer Center, ul. Roentgena 5, Warsaw, Poland. Tel.: +48 225462728. E-mail address: [email protected] (M. Rysz). 0030-6657/$ – see front matter © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
otolaryngologia polska 68 (2014) 42–45
Surgical biopsy of the tumor was made. Pathological examination revealed spindle-shaped cells visible in H-E stain. In immunohistochemical analysis Smoth Muscle Actin and caldesmon antigens were positive. Desmin antigen immunoreactivity was negative. These findings suggested diagnosis of LMS. The patient underwent surgical excision of the tumor under local anesthesia (Fig. 1). The tumor was removed using wedge-resection technique, with auricle cartilage and skin, with margin of healthy tissues. Postoperative pathological examination showed that closest surgical margin was 0.7 cm. In view of these findings adjuvant therapy was not indicated. The patient has been followed-up for 3 years without signs of recurrence.
Discussion LMS is classified as a soft tissue sarcoma occurring mostly among adults aged 40–60 years, more frequently in men [3, 5]. Sarcomas are malignant, aggressive neoplasms and LMS, which belongs to this group, can be found in head and neck region as a metastasis from other part of the body. In the case reported in this paper, the LMS developed as primary tumor in the auricle. In available literature LMS cases have been described within several head and neck locations. LMS is described as an exophytic, painless mass. Border between tumor and skin is irregular but well defined. There are neither ulcerations nor nodules near the tumor. Metastases to the local lymph nodes are absent. Observations in our case confirm presence of the same features. Origin of the primary focus of LMS in head and neck region is a very interesting issue. It has been suggested that smooth muscles of the vessels walls are probably the tissue where LMS develops. But LMS primary focus could be as well in smooth
43
muscles of the hair follicle, salivary glands cells, or circumvallated papillae’s in cases where LMS was found in the tongue [1, 6–8]. Some authors distinguish different place of origin for different LMS types – cutaneous leiomyosarcoma develops from erector pile muscles in hair follicle whereas subcutaneous leiomyosarcoma develops from the smooth muscle of the wall of small or medium sized veins. In our opinion, the most likely origin of the LMS in reported case are smooth muscles of the blood vessels walls, as there was no evidence of skin infiltration. For histopathological diagnosis of the LMS the immunohistochemical examination is necessary [1, 4, 5, 8]. Although LMS of the head and neck, especially of the external ear, are rare, they present the same morphological features as lesions encountered in other soft tissue sites. Histologically, the tumors show poorly differentiated, atypical spindle-shaped cells with elongated nuclei and blunted ends, arranged in fascicles. This pattern may be less well defined in some tumors, and occasionally there is a focal storiform, palisaded, or haemangiopericytoma-like arrangement. The tumors are usually compactly cellular, but fibrosis or myxoid change may be present. Hyalinized, hypocellular zones and necrosis are frequent in larger leiomyosarcoma. Nuclear pleomorphism is generally notable, although it may be focal, mild, or occasionally absent. Mitotic figures can usually be found readily, although they may be few or patchy, and atypical mitoses are often seen. Smooth muscle actin, desmin and h-caldesmon are positive in a great majority of soft tissue leiomyosarcoma. However, none of these is absolutely specific for smooth muscle and positivity for two of these markers is more supportive of leiomyosarcoma than positivity for one alone. ‘‘Dedifferentiated’’ areas may be negative for SMA and desmin, but total negativity for both in a tumor would cast great doubt on the diagnosis of leiomyosarcoma (Figs. 2–5).
Fig. 1 – On the left – patient before operation, on the right – the same patient two weeks after tumor resection
44
otolaryngologia polska 68 (2014) 42–45
Fig. 2 – Cutaneous leiomosarcoma showing typical intersecting groups of spindle cells
Fig. 4 – Tumor cells show focal reactivity for desmin
Fig. 3 – LMS composed of eosinophilic spindle cells with focal nuclear hyperchromatism and pleomorphism
Fig. 5 – Tumor cells show strong reactivity for SMA
In general, the diagnosis of soft tissue leiomyosarcoma should not be made on the basis of immunostains in the absence of appropriate morphologic features. The reported cases of the primary LMS in head and neck region were localized in the mouth mucosa – 3 [1], tongue – 6 [5], parapharyngeal space – 2 [9], scalp skin – 1 [2], thyroid gland – 6 [8], maxilla – 3, palate – 2, and in mandible – 3 [5]. The most frequent localization of LMS in head and neck region is larynx, and some 45 cases have been reported [9]. In about 50% of the reported cases, there was local recurrence or distant metastases [3, 5]. In bibliography, we have not found correlation between size or location of the tumor and recurrence. In all head and neck sarcomas overall 5 year survival rate ranges from 32% to 87% and 5 year disease-free survival rates range from 27% to 66% in different cancer centers and different sarcoma subgroups [10]. The most successful therapy for soft tissue sarcomas is surgical excision [1, 3, 8, 13]. The advised margin of the healthy
tissues has to be from 1 to 5 cm around the tumor [3, 7, 9]. Adequate surgical resection of LMS is directly associated with long term survival rate [5, 6]. Removal of cervical lymph nodes is not advised [3, 7]. Value of the radiotherapy (RTH) in management of LMS is doubtful [3, 6, 9]. There are no large, randomized trials of the use of RTH in soft tissue sarcomas of the head and neck region [9]. Additionally, LMS can develop after RTH [11, 12]. In studies concerning soft tissue sarcomas therapy, the survival rate was worse in patients after surgical excision and RTH, as compared to the group treated only by surgery [10]. Since adjuvant RTH is usually advised in cases where surgical margins are small or inadequate, importance of these findings is unclear. Chemotherapy in LMS is considered ineffective, and it is used only in palliative treatment [3, 6, 13]. Surgical excision of the LMS, as compared to radio- or chemotherapy, is the best therapeutic option. Great attention should be paid to appropriate resection of head and neck sarcomas including LMS, although available literature, and
otolaryngologia polska 68 (2014) 42–45
our case, does not provide clear indication how large surgical margins should be.
Authors’ contributions/Wkład autoro´w According to order.
Conflict of interest/Konflikt interesu None declared.
Financial support/Finansowanie None declared.
Ethics/Etyka The work described in this article have been carried out in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki) for experiments involving humans; EU Directive 2010/63/EU for animal experiments; Uniform Requirements for manuscripts submitted to Biomedical journals. The own research were conducted according to the Good Clinical Practice guidelines and accepted by local Bioethics Committee, all patients agreed in writing to participation and these researches.
r e f e r e n c e s / p i s´ m i e n n i c t w o
[1] Akcam T, Oysul K, Birkent H, Gerek M, Yetiser S. Leiomyosarcoma of the head and neck: report of two cases and review of the literature. Auris Nasus Larynx 2005;32:209–212.
45
[2] Batra PS, Kern RC, Pelzer HJ, Heines 3rd GK. Leiomyosarcoma of the sinonasal tract: report of a case. Otolaryngology – Head and Neck Surgery 2001;125(6):663–664. [3] Demirkan F, Unal S, Cenetoqlu S, Cinel L. Radiationinduced leiomyosarcomas as second primary tumors in the head and neck region: report of 2 cases. Journal of Oral and Maxillofacial Surgery 2003;61(2):259–263. [4] Jay A, Piper K, Farthing PM, Carter J, Diwakar A. Low-grade myofibroblastic sarcoma of the tongue. Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontics 2007;104:e52–e58. [5] Lajer CB, Daugaard S, Hansen HS, Kirkegaard J, Holmgaard S, Christensen ME. Soft tissue sarcomas of the head and neck: a single-centre experience. Clinical Otolaryngology 2005;30:176–182. [6] Montgomery E, Goldblum JR, Fisher C. Leiomyosarcoma of the head and neck: a clinicopathological study. Histopathology 2002;40:518–525. [7] Oztrk K, Keles B, Arbaq H, Yo¨ndemll F, Avunduk MC. Postauricular subcutaneous leiomyosarcoma. Auris Nasus Larynx 2004;31(3):323–328. [8] Pai SI, Limb CJ, Nicol T, Williams MF. Leiomyosarcoma of the auricle. Otolaryngology – Head and Neck Surgery 2003;128(3):442–444. [9] Premalata CS, Kumar RV, Malathi M, Shenoy AM, Nanjundappa N. Cutaneous leiomyosarcoma, trichoblastoma, and syringocystadenoma papilliferum arising from nevus sebaceous. International Journal of Dermatology 2007;46(3):306–308. [10] Sedghizadeh PP, Angiero F, Allen CM, Kalmar JR, Rawal Y, Albright EA. Post-irradiation leiomyosarcoma of the maxilla: report of a case in a patient with prior radiation treatment for retinoblastoma. Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontics 2004;97:726–731. [11] Thompson LD, Wenig BM, Adair CF, Shmookler BM, Heffess CS. Primary smooth muscle tumors of the thyroid gland. Cancer 1997;79(3):579–587. [12] Yadav R, Bharthan S. Leiomyosarcoma of the buccal mucosa: a case report with immunochemitry findings. Journal of Oral Science 2008;50(2):215–218. [13] Yang SW, Chen TM, Tsai CY, Lin CY. A peculiar site of leiomyosarcoma: the tongue tip – report of a case. International Journal of Oral and Maxillofacial Surgery 2006;35:469–471.
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/otpol
Case report/Kazuistyka
Leiomyosarcoma of the external ear – Case report Mie˛sak gładkokomo´ rkowy ucha – opis przypadku Maciej Rysz 1,*, Janusz Jaworowski 1, Romuald Krajewski 1, Joanna Baran´ ska 2 1
Head and Neck Cancer Department, Surgery Division, Memorial Cancer Center, Head: A. Kawecki MD, PhD, Poland 2 Department of Pathology, Memorial Cancer Center, Warsaw, Head: K. Ptaszyn´ ski MD, Poland
article info
abstract
Article history:
In head and neck region leiomyosarcoma is a very rare finding. This tumor is usually located
Received: 29.01.2012
in abdominal cavity. We report a case of 78-year-old man with tumor of the auricle that was
Accepted: 13.06.2012
resected with 0.7 cm margin. Histological and immunohistochemical analysis revealed the
Available online: 18.06.2012
leiomyosarcoma. Adjuvant therapy was not advised. During 3 years of follow-up there are no signs of recurrence. Review of the literature shows that the survival of patients with leiomyosarcoma in head and neck region seems to be similar to other kinds of sarcomas in
Keywords: Leiomiosarcoma
this localization. It is not clear, how large surgical margins should be to achieve satisfactory
Sarcomas of the head
local control. © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by
Słowa kluczowe: mie˛sak gładkokomo´rkowy mie˛saki głowy
Elsevier Urban & Partner Sp. z o.o. All rights reserved.
Introduction
Case report
Leiomyosarcoma (LMS) is a malignant mesenchymal neoplasm of smooth muscles origin [1]. Two types of LMS – superficial and cutaneous are distinguished. Cutaneous leiomyosarcoma grows slowly, and has better prognosis than subcutaneous leiomyosarcoma [1, 2]. LMS is diagnosed in approximately 6% of all soft tissues sarcomas, and head and neck region sarcomas constitute less than 1% of tumors in this region [1, 3]. Most frequently, LMS is found in uterus, gastrointestinal tract and retroperitoneal space. Location in head and neck region is very rare [4]. We report an extremely rare case of LMS of external ear.
The patient is a 78-year-old man with rapidly growing tumor on the left auricle. A small spot, less than 1 cm in diameter on the ear skin has been observed since about 5 years. Two months before admission the tumor started to grow rapidly. The examination revealed an exophytic tumor on the left auricle measuring 1 1.5 1.5 cm. It was attached to the auricle cartilage, but well separated from the surrounding skin. One centimeter skin margin around the tumor was reddened, painless, without palpable infiltration. There was no evidence of cervical node or distant metastases (T1, N0, M0).
* Corresponding author: Maciej Rysz, Head and Neck Department, Cancer Center, ul. Roentgena 5, Warsaw, Poland. Tel.: +48 225462728. E-mail address: [email protected] (M. Rysz). 0030-6657/$ – see front matter © 2012 Polish Otorhinolaryngology - Head and Neck Surgery Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
otolaryngologia polska 68 (2014) 42–45
Surgical biopsy of the tumor was made. Pathological examination revealed spindle-shaped cells visible in H-E stain. In immunohistochemical analysis Smoth Muscle Actin and caldesmon antigens were positive. Desmin antigen immunoreactivity was negative. These findings suggested diagnosis of LMS. The patient underwent surgical excision of the tumor under local anesthesia (Fig. 1). The tumor was removed using wedge-resection technique, with auricle cartilage and skin, with margin of healthy tissues. Postoperative pathological examination showed that closest surgical margin was 0.7 cm. In view of these findings adjuvant therapy was not indicated. The patient has been followed-up for 3 years without signs of recurrence.
Discussion LMS is classified as a soft tissue sarcoma occurring mostly among adults aged 40–60 years, more frequently in men [3, 5]. Sarcomas are malignant, aggressive neoplasms and LMS, which belongs to this group, can be found in head and neck region as a metastasis from other part of the body. In the case reported in this paper, the LMS developed as primary tumor in the auricle. In available literature LMS cases have been described within several head and neck locations. LMS is described as an exophytic, painless mass. Border between tumor and skin is irregular but well defined. There are neither ulcerations nor nodules near the tumor. Metastases to the local lymph nodes are absent. Observations in our case confirm presence of the same features. Origin of the primary focus of LMS in head and neck region is a very interesting issue. It has been suggested that smooth muscles of the vessels walls are probably the tissue where LMS develops. But LMS primary focus could be as well in smooth
43
muscles of the hair follicle, salivary glands cells, or circumvallated papillae’s in cases where LMS was found in the tongue [1, 6–8]. Some authors distinguish different place of origin for different LMS types – cutaneous leiomyosarcoma develops from erector pile muscles in hair follicle whereas subcutaneous leiomyosarcoma develops from the smooth muscle of the wall of small or medium sized veins. In our opinion, the most likely origin of the LMS in reported case are smooth muscles of the blood vessels walls, as there was no evidence of skin infiltration. For histopathological diagnosis of the LMS the immunohistochemical examination is necessary [1, 4, 5, 8]. Although LMS of the head and neck, especially of the external ear, are rare, they present the same morphological features as lesions encountered in other soft tissue sites. Histologically, the tumors show poorly differentiated, atypical spindle-shaped cells with elongated nuclei and blunted ends, arranged in fascicles. This pattern may be less well defined in some tumors, and occasionally there is a focal storiform, palisaded, or haemangiopericytoma-like arrangement. The tumors are usually compactly cellular, but fibrosis or myxoid change may be present. Hyalinized, hypocellular zones and necrosis are frequent in larger leiomyosarcoma. Nuclear pleomorphism is generally notable, although it may be focal, mild, or occasionally absent. Mitotic figures can usually be found readily, although they may be few or patchy, and atypical mitoses are often seen. Smooth muscle actin, desmin and h-caldesmon are positive in a great majority of soft tissue leiomyosarcoma. However, none of these is absolutely specific for smooth muscle and positivity for two of these markers is more supportive of leiomyosarcoma than positivity for one alone. ‘‘Dedifferentiated’’ areas may be negative for SMA and desmin, but total negativity for both in a tumor would cast great doubt on the diagnosis of leiomyosarcoma (Figs. 2–5).
Fig. 1 – On the left – patient before operation, on the right – the same patient two weeks after tumor resection
44
otolaryngologia polska 68 (2014) 42–45
Fig. 2 – Cutaneous leiomosarcoma showing typical intersecting groups of spindle cells
Fig. 4 – Tumor cells show focal reactivity for desmin
Fig. 3 – LMS composed of eosinophilic spindle cells with focal nuclear hyperchromatism and pleomorphism
Fig. 5 – Tumor cells show strong reactivity for SMA
In general, the diagnosis of soft tissue leiomyosarcoma should not be made on the basis of immunostains in the absence of appropriate morphologic features. The reported cases of the primary LMS in head and neck region were localized in the mouth mucosa – 3 [1], tongue – 6 [5], parapharyngeal space – 2 [9], scalp skin – 1 [2], thyroid gland – 6 [8], maxilla – 3, palate – 2, and in mandible – 3 [5]. The most frequent localization of LMS in head and neck region is larynx, and some 45 cases have been reported [9]. In about 50% of the reported cases, there was local recurrence or distant metastases [3, 5]. In bibliography, we have not found correlation between size or location of the tumor and recurrence. In all head and neck sarcomas overall 5 year survival rate ranges from 32% to 87% and 5 year disease-free survival rates range from 27% to 66% in different cancer centers and different sarcoma subgroups [10]. The most successful therapy for soft tissue sarcomas is surgical excision [1, 3, 8, 13]. The advised margin of the healthy
tissues has to be from 1 to 5 cm around the tumor [3, 7, 9]. Adequate surgical resection of LMS is directly associated with long term survival rate [5, 6]. Removal of cervical lymph nodes is not advised [3, 7]. Value of the radiotherapy (RTH) in management of LMS is doubtful [3, 6, 9]. There are no large, randomized trials of the use of RTH in soft tissue sarcomas of the head and neck region [9]. Additionally, LMS can develop after RTH [11, 12]. In studies concerning soft tissue sarcomas therapy, the survival rate was worse in patients after surgical excision and RTH, as compared to the group treated only by surgery [10]. Since adjuvant RTH is usually advised in cases where surgical margins are small or inadequate, importance of these findings is unclear. Chemotherapy in LMS is considered ineffective, and it is used only in palliative treatment [3, 6, 13]. Surgical excision of the LMS, as compared to radio- or chemotherapy, is the best therapeutic option. Great attention should be paid to appropriate resection of head and neck sarcomas including LMS, although available literature, and
otolaryngologia polska 68 (2014) 42–45
our case, does not provide clear indication how large surgical margins should be.
Authors’ contributions/Wkład autoro´w According to order.
Conflict of interest/Konflikt interesu None declared.
Financial support/Finansowanie None declared.
Ethics/Etyka The work described in this article have been carried out in accordance with The Code of Ethics of the World Medical Association (Declaration of Helsinki) for experiments involving humans; EU Directive 2010/63/EU for animal experiments; Uniform Requirements for manuscripts submitted to Biomedical journals. The own research were conducted according to the Good Clinical Practice guidelines and accepted by local Bioethics Committee, all patients agreed in writing to participation and these researches.
r e f e r e n c e s / p i s´ m i e n n i c t w o
[1] Akcam T, Oysul K, Birkent H, Gerek M, Yetiser S. Leiomyosarcoma of the head and neck: report of two cases and review of the literature. Auris Nasus Larynx 2005;32:209–212.
45
[2] Batra PS, Kern RC, Pelzer HJ, Heines 3rd GK. Leiomyosarcoma of the sinonasal tract: report of a case. Otolaryngology – Head and Neck Surgery 2001;125(6):663–664. [3] Demirkan F, Unal S, Cenetoqlu S, Cinel L. Radiationinduced leiomyosarcomas as second primary tumors in the head and neck region: report of 2 cases. Journal of Oral and Maxillofacial Surgery 2003;61(2):259–263. [4] Jay A, Piper K, Farthing PM, Carter J, Diwakar A. Low-grade myofibroblastic sarcoma of the tongue. Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontics 2007;104:e52–e58. [5] Lajer CB, Daugaard S, Hansen HS, Kirkegaard J, Holmgaard S, Christensen ME. Soft tissue sarcomas of the head and neck: a single-centre experience. Clinical Otolaryngology 2005;30:176–182. [6] Montgomery E, Goldblum JR, Fisher C. Leiomyosarcoma of the head and neck: a clinicopathological study. Histopathology 2002;40:518–525. [7] Oztrk K, Keles B, Arbaq H, Yo¨ndemll F, Avunduk MC. Postauricular subcutaneous leiomyosarcoma. Auris Nasus Larynx 2004;31(3):323–328. [8] Pai SI, Limb CJ, Nicol T, Williams MF. Leiomyosarcoma of the auricle. Otolaryngology – Head and Neck Surgery 2003;128(3):442–444. [9] Premalata CS, Kumar RV, Malathi M, Shenoy AM, Nanjundappa N. Cutaneous leiomyosarcoma, trichoblastoma, and syringocystadenoma papilliferum arising from nevus sebaceous. International Journal of Dermatology 2007;46(3):306–308. [10] Sedghizadeh PP, Angiero F, Allen CM, Kalmar JR, Rawal Y, Albright EA. Post-irradiation leiomyosarcoma of the maxilla: report of a case in a patient with prior radiation treatment for retinoblastoma. Oral Surgery Oral Medicine Oral Pathology Oral Radiology and Endodontics 2004;97:726–731. [11] Thompson LD, Wenig BM, Adair CF, Shmookler BM, Heffess CS. Primary smooth muscle tumors of the thyroid gland. Cancer 1997;79(3):579–587. [12] Yadav R, Bharthan S. Leiomyosarcoma of the buccal mucosa: a case report with immunochemitry findings. Journal of Oral Science 2008;50(2):215–218. [13] Yang SW, Chen TM, Tsai CY, Lin CY. A peculiar site of leiomyosarcoma: the tongue tip – report of a case. International Journal of Oral and Maxillofacial Surgery 2006;35:469–471.
![[Leiomyosarcoma of the external ear canal].](/assets/img/hold.png)
