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An acquired tufted angioma of the nasal cavity
Auris Nasus Larynx 40 (2013) 581–583
Contents lists available at SciVerse ScienceDirect
Auris Nasus Larynx journal homepage: www.elsevier.com/locate/anl
An acquired tufted angioma of the nasal cavity Young Ha Kim, Guen-Ho Lee, Su Hyun Lee, Jin Hee Cho * Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, The Catholic University of Korea, Seoul, South Korea
A R T I C L E I N F O
A B S T R A C T
Article history: Received 24 August 2012 Accepted 29 January 2013 Available online 18 March 2013
Tufted angiomas are benign vascular tumors that occur mainly in children younger than 5 years, involving the skin with subcutaneous plaques or in a nodular form. We experienced a rare tufted angioma in the nasal cavity. A 35-year-old woman visited our clinic because of frequent epistaxis. A mass was found in the left posterior nasal cavity, adjacent to the middle turbinate, and attached to the nasal septum. The mass was excised using endoscopy, under local anesthesia. The histopathological examination showed a cannon-ball distribution of the vasculature, compatible with a tufted angioma. This is the first tufted angioma in the English literature found in the nasal mucosa, so we report this case with a literature review. ß 2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Tufted angioma Nasal cavity Epistaxis Endoscopic surgery
1. Introduction A tufted angioma (TA) is a rare, benign, vascular tumor, first described in 1949 as an angioblastoma [1]. Tufted angiomas involve mainly the skin or subcutaneous layer in the form of an erythematous macule, occurring primarily on the neck, upper trunk, and extremities of children [1]. There are several reports of TAs in the oral mucosa [2,3] or perianal area [4], but these are extremely rare. Most cases (60–70%) of TA develop before the age of 5 years, and fewer than 10% occur after the age of 50 years. [5]. Tufted angioma was named after the histopathological finding of cannon ball-shaped vascular nests. Clinically, TAs are expressed as erythematous macules or deep subcutaneous plaques, and there are no reports of a TA seen in the form of a mass. We report a mass-like TA found in the left nasal cavity of a 36year-old female who complained of frequent epistaxis and nasal stuffiness. The mass was removed surgically using endoscopy and diagnosed as a TA histologically. No TA in a pure mucosal surface has been reported in the English-language literature.
2. Case report A 36-year-old, healthy, nongravid woman came to the rhinology clinic with left dominant epistaxis and nasal stuffiness for 6 months. Her medical and family histories were non-
* Corresponding author at: Yeouido St. Mary’s Hospital, 62 Yeouidodong, Yeongdeungpo, Seoul 150-713, South Korea. Tel.: +82 2 3779 2048; fax: +82 2 786 1149. E-mail address: [email protected] (J.H. Cho). 0385-8146/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.anl.2013.01.004
contributory. Upon examination of the nasal cavity, a 1.0 1.5cm, round, smooth-surfaced mass that tended to bleed easily was found in the left posterior nasal cavity. The mass was attached to the nasal septum at the level of the middle turbinate inferior end and it was blocking the superior 1/3 of the posterior choana (Fig. 1). The hematological examination was normal and the mass originated from the nasal septum with a stalk, not the lateral nasal wall. The presumptive diagnosis was a hemangioma, with a small likelihood it being an angiofibroma. The mass was excised using endoscopy under local anesthesia with no complications. The origin in the mucosa was clear, with no bleeding, and the patient was discharged the day of the operation. Ten days postoperatively, the mucosal surface of the nasal cavity where the mass had been was healed. Pathologically, scattered round and ovoid dermal lobules in a ‘‘cannonball’’ distribution were present (Fig. 2A). These tufts were composed primarily of plump endothelial cells, with interspersed narrow lymphatic-like channels (Fig. 2B). The tumor showed inmunopositivity for CD34 and alpha-actin (Fig. 3A and B) and immunonegativity for GLUT-1 (Fig. 3C). From these findings, the final diagnosis of a tufted angioma was made. The preoperative complaints had all resolved at follow-up 6 months postoperatively, with no signs of recurrence. 3. Discussion Tufted angiomas are usually found on the skin, or rarely on a mucosal surface, as a superficial nodular lesion, measuring 2– 5 cm2 [3,5,6]. TAs are diagnosed mainly in children younger than 5 years old, while about 30% of cases are diagnosed in adults. Although the causes of TA are not clear, relationships with pregnancy [4], trauma [7], immunosuppression [5], or solid organ
582
Y.H. Kim et al. / Auris Nasus Larynx 40 (2013) 581–583
Fig. 1. Preoperative endoscopy of the left nasal cavity showed a smooth-surfaced, round mass in the posterior nasal cavity, just adjacent to the middle turbinate. The mass was blocking the upper 1/3 of the posterior choana and its stalk was attached to the posterior nasal septum.
Fig. 3. Immunostaining for CD34 (A) and alpha-actin (B) was positive and that for GLUT-1 (C) was negative (original 400 magnification).
Fig. 2. (A) A hematoxylin-eosin stained specimen at 100 magnification showed scattered round and ovoid dermal lobules called tufts in a ‘‘cannonball’’ distribution (black arrows). (B) High magnification of lobules shows (400 magnification) spindle cells separated by lumina containing few red blood cells (star), surrounded by dilated, crescent-shaped, vascular channels. No mitotic activity or cellular atypia were seen.
transplantation [8] have been reported. In this case, the only notable finding was that the patient was a female of childbearing age. The differential diagnosis includes pyogenic granuloma, hemangioma of infancy, port-wine stain, vascular malformation,
bacillary angiomatosis, lymphangioma, hemangiopericytoma, Kaposi’s sarcoma, and Kaposiform hemangioendothelioma [5]. An annular type TA should be differentiated from lupus vulgaris, annular sarcoid, or granuloma annulare [6]. The diagnosis should consider the patient’s age, and location or shape of the lesion, but the ultimate method of diagnosis is pathological confirmation. Previous studies of TA in children describe a male predominance (69%) [9]. However, a thorough review of 22 case reports of TA in adults [2,3,5–8], showed a clear female predominance, with a male: female ratio of 15:7 (68%) and a mean age at diagnosis of 44.3 years. Our case was similar demographically. The neck, upper trunk, and shoulders [3] are the most commonly reported sites of TA. Other involved sites, such as the face, scalp, and proximal extremities [5] are uncommon. Few
Y.H. Kim et al. / Auris Nasus Larynx 40 (2013) 581–583
reported cases have involved mucosal surfaces, such as the oral mucosa [3] and perianal area [4]. Strictly speaking, these reported mucosal surfaces were at the skin-mucosal border. In this case, the TA was located in the nasal cavity near the posterior choana, which is a pure mucosal surface, and it was a mass, which has to our knowledge never been reported. No treatment guidelines for TA have been established, so the patient’s age, location of the lesion, and symptoms should be considered when determining the treatment. Congenital TA or TA in children [9], cases related to pregnancy [4], and small, asymptomatic lesions with no cosmetic problems [3] can be monitored and spontaneous regression is likely. However, a huge lesion or a TA in a child combined with thrombocytopenic coagulopathy, i.e., Kasabach–Merritt syndrome, should be treated [9]. If the lesion is painful or growing, medical therapy with oral or topical steroids can be used to minimize the symptoms and size. If the response to steroids is limited, vincristine or interferon-a is useful [3,10]. In cases that are localized or involve esthetic or functional problems, surgical excision is appropriate [3,4]. Regular follow-up is necessary since recurrence is common after surgery. In our case, as the epistaxis was obvious, the lesion was localized, and was in mass form, endoscopic excision was successful. Regular follow up is needed due to the possibility of recurrence. 4. Conclusion We present the first case of a TA in the nasal cavity. TA in a pure mucosal surface is extremely rare. Complete surgical resection was
583
performed with good clinical results. Regular follow-up is necessary since TAs tend to recur. Conflict of interest None. References [1] Jones EW, Orkin M. Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi’s sarcoma or low-grade angiosarcoma. J Am Acad Dermatol 1989;20:214–25. [2] Kleinegger CL, Hammond HL, Vincent SD, Finkelstein MW. Acquired tufted angioma: a unique vascular lesion not previously reported in the oral mucosa. Br J Dermatol 2000;142:794–9. [3] Lee B, Chiu M, Soriano T, Craft N. Adult-onset tufted angioma: a case report and review of the literature. Cutis 2006;78:341–5. [4] Pietroletti R, Leardi S, Simi M. Perianal acquired tufted angioma associated with pregnancy: case report. Tech Coloproctol 2002;6:117–9. [5] Ghosh SK, Bandyopadhyay D, Ghosh A, Biswas SK, Barma KD. Acquired multifocal tufted angiomas in an immunocompetent young adult. Indian J Dermatol 2011;56:412–4. [6] Chakraborty S, Gharami RC, Das NK, Datta PK. Annular tufted angioma: a rare entity. Int J Dermatol 2009;48:614–6. [7] Silva RS, Bressan AL, Nascimento LB, Kac BK, Azulay-Abulafia L. Tufted angioma and myofascial pain syndrome. An Bras Dermatol 2011;86:125–7. [8] Kim CY, Nam YH, Kim GD, Oh CW. Tufted angioma in site of healed herpes zoster: isotopic response. Clin Exp Dermatol 2006;31:714–5. [9] Osio A, Fraitag S, Hadj-Rabia S, Bodemer C, de Prost Y, Hamel-Teillac D. Clinical spectrum of tufted angiomas in childhood: a report of 13 cases and a review of the literature. Arch Dermatol 2010;146:758–63. [10] Ferrandiz-Pulido C, Mollet J, Sabado C, Ferrer B, Garcia-Patos V. Tufted angioma associated with Kasabach–Merritt phenomenon: a therapeutic challenge. Acta Derm Venereol 2010;90:535–7.
Contents lists available at SciVerse ScienceDirect
Auris Nasus Larynx journal homepage: www.elsevier.com/locate/anl
An acquired tufted angioma of the nasal cavity Young Ha Kim, Guen-Ho Lee, Su Hyun Lee, Jin Hee Cho * Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, The Catholic University of Korea, Seoul, South Korea
A R T I C L E I N F O
A B S T R A C T
Article history: Received 24 August 2012 Accepted 29 January 2013 Available online 18 March 2013
Tufted angiomas are benign vascular tumors that occur mainly in children younger than 5 years, involving the skin with subcutaneous plaques or in a nodular form. We experienced a rare tufted angioma in the nasal cavity. A 35-year-old woman visited our clinic because of frequent epistaxis. A mass was found in the left posterior nasal cavity, adjacent to the middle turbinate, and attached to the nasal septum. The mass was excised using endoscopy, under local anesthesia. The histopathological examination showed a cannon-ball distribution of the vasculature, compatible with a tufted angioma. This is the first tufted angioma in the English literature found in the nasal mucosa, so we report this case with a literature review. ß 2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Tufted angioma Nasal cavity Epistaxis Endoscopic surgery
1. Introduction A tufted angioma (TA) is a rare, benign, vascular tumor, first described in 1949 as an angioblastoma [1]. Tufted angiomas involve mainly the skin or subcutaneous layer in the form of an erythematous macule, occurring primarily on the neck, upper trunk, and extremities of children [1]. There are several reports of TAs in the oral mucosa [2,3] or perianal area [4], but these are extremely rare. Most cases (60–70%) of TA develop before the age of 5 years, and fewer than 10% occur after the age of 50 years. [5]. Tufted angioma was named after the histopathological finding of cannon ball-shaped vascular nests. Clinically, TAs are expressed as erythematous macules or deep subcutaneous plaques, and there are no reports of a TA seen in the form of a mass. We report a mass-like TA found in the left nasal cavity of a 36year-old female who complained of frequent epistaxis and nasal stuffiness. The mass was removed surgically using endoscopy and diagnosed as a TA histologically. No TA in a pure mucosal surface has been reported in the English-language literature.
2. Case report A 36-year-old, healthy, nongravid woman came to the rhinology clinic with left dominant epistaxis and nasal stuffiness for 6 months. Her medical and family histories were non-
* Corresponding author at: Yeouido St. Mary’s Hospital, 62 Yeouidodong, Yeongdeungpo, Seoul 150-713, South Korea. Tel.: +82 2 3779 2048; fax: +82 2 786 1149. E-mail address: [email protected] (J.H. Cho). 0385-8146/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.anl.2013.01.004
contributory. Upon examination of the nasal cavity, a 1.0 1.5cm, round, smooth-surfaced mass that tended to bleed easily was found in the left posterior nasal cavity. The mass was attached to the nasal septum at the level of the middle turbinate inferior end and it was blocking the superior 1/3 of the posterior choana (Fig. 1). The hematological examination was normal and the mass originated from the nasal septum with a stalk, not the lateral nasal wall. The presumptive diagnosis was a hemangioma, with a small likelihood it being an angiofibroma. The mass was excised using endoscopy under local anesthesia with no complications. The origin in the mucosa was clear, with no bleeding, and the patient was discharged the day of the operation. Ten days postoperatively, the mucosal surface of the nasal cavity where the mass had been was healed. Pathologically, scattered round and ovoid dermal lobules in a ‘‘cannonball’’ distribution were present (Fig. 2A). These tufts were composed primarily of plump endothelial cells, with interspersed narrow lymphatic-like channels (Fig. 2B). The tumor showed inmunopositivity for CD34 and alpha-actin (Fig. 3A and B) and immunonegativity for GLUT-1 (Fig. 3C). From these findings, the final diagnosis of a tufted angioma was made. The preoperative complaints had all resolved at follow-up 6 months postoperatively, with no signs of recurrence. 3. Discussion Tufted angiomas are usually found on the skin, or rarely on a mucosal surface, as a superficial nodular lesion, measuring 2– 5 cm2 [3,5,6]. TAs are diagnosed mainly in children younger than 5 years old, while about 30% of cases are diagnosed in adults. Although the causes of TA are not clear, relationships with pregnancy [4], trauma [7], immunosuppression [5], or solid organ
582
Y.H. Kim et al. / Auris Nasus Larynx 40 (2013) 581–583
Fig. 1. Preoperative endoscopy of the left nasal cavity showed a smooth-surfaced, round mass in the posterior nasal cavity, just adjacent to the middle turbinate. The mass was blocking the upper 1/3 of the posterior choana and its stalk was attached to the posterior nasal septum.
Fig. 3. Immunostaining for CD34 (A) and alpha-actin (B) was positive and that for GLUT-1 (C) was negative (original 400 magnification).
Fig. 2. (A) A hematoxylin-eosin stained specimen at 100 magnification showed scattered round and ovoid dermal lobules called tufts in a ‘‘cannonball’’ distribution (black arrows). (B) High magnification of lobules shows (400 magnification) spindle cells separated by lumina containing few red blood cells (star), surrounded by dilated, crescent-shaped, vascular channels. No mitotic activity or cellular atypia were seen.
transplantation [8] have been reported. In this case, the only notable finding was that the patient was a female of childbearing age. The differential diagnosis includes pyogenic granuloma, hemangioma of infancy, port-wine stain, vascular malformation,
bacillary angiomatosis, lymphangioma, hemangiopericytoma, Kaposi’s sarcoma, and Kaposiform hemangioendothelioma [5]. An annular type TA should be differentiated from lupus vulgaris, annular sarcoid, or granuloma annulare [6]. The diagnosis should consider the patient’s age, and location or shape of the lesion, but the ultimate method of diagnosis is pathological confirmation. Previous studies of TA in children describe a male predominance (69%) [9]. However, a thorough review of 22 case reports of TA in adults [2,3,5–8], showed a clear female predominance, with a male: female ratio of 15:7 (68%) and a mean age at diagnosis of 44.3 years. Our case was similar demographically. The neck, upper trunk, and shoulders [3] are the most commonly reported sites of TA. Other involved sites, such as the face, scalp, and proximal extremities [5] are uncommon. Few
Y.H. Kim et al. / Auris Nasus Larynx 40 (2013) 581–583
reported cases have involved mucosal surfaces, such as the oral mucosa [3] and perianal area [4]. Strictly speaking, these reported mucosal surfaces were at the skin-mucosal border. In this case, the TA was located in the nasal cavity near the posterior choana, which is a pure mucosal surface, and it was a mass, which has to our knowledge never been reported. No treatment guidelines for TA have been established, so the patient’s age, location of the lesion, and symptoms should be considered when determining the treatment. Congenital TA or TA in children [9], cases related to pregnancy [4], and small, asymptomatic lesions with no cosmetic problems [3] can be monitored and spontaneous regression is likely. However, a huge lesion or a TA in a child combined with thrombocytopenic coagulopathy, i.e., Kasabach–Merritt syndrome, should be treated [9]. If the lesion is painful or growing, medical therapy with oral or topical steroids can be used to minimize the symptoms and size. If the response to steroids is limited, vincristine or interferon-a is useful [3,10]. In cases that are localized or involve esthetic or functional problems, surgical excision is appropriate [3,4]. Regular follow-up is necessary since recurrence is common after surgery. In our case, as the epistaxis was obvious, the lesion was localized, and was in mass form, endoscopic excision was successful. Regular follow up is needed due to the possibility of recurrence. 4. Conclusion We present the first case of a TA in the nasal cavity. TA in a pure mucosal surface is extremely rare. Complete surgical resection was
583
performed with good clinical results. Regular follow-up is necessary since TAs tend to recur. Conflict of interest None. References [1] Jones EW, Orkin M. Tufted angioma (angioblastoma). A benign progressive angioma, not to be confused with Kaposi’s sarcoma or low-grade angiosarcoma. J Am Acad Dermatol 1989;20:214–25. [2] Kleinegger CL, Hammond HL, Vincent SD, Finkelstein MW. Acquired tufted angioma: a unique vascular lesion not previously reported in the oral mucosa. Br J Dermatol 2000;142:794–9. [3] Lee B, Chiu M, Soriano T, Craft N. Adult-onset tufted angioma: a case report and review of the literature. Cutis 2006;78:341–5. [4] Pietroletti R, Leardi S, Simi M. Perianal acquired tufted angioma associated with pregnancy: case report. Tech Coloproctol 2002;6:117–9. [5] Ghosh SK, Bandyopadhyay D, Ghosh A, Biswas SK, Barma KD. Acquired multifocal tufted angiomas in an immunocompetent young adult. Indian J Dermatol 2011;56:412–4. [6] Chakraborty S, Gharami RC, Das NK, Datta PK. Annular tufted angioma: a rare entity. Int J Dermatol 2009;48:614–6. [7] Silva RS, Bressan AL, Nascimento LB, Kac BK, Azulay-Abulafia L. Tufted angioma and myofascial pain syndrome. An Bras Dermatol 2011;86:125–7. [8] Kim CY, Nam YH, Kim GD, Oh CW. Tufted angioma in site of healed herpes zoster: isotopic response. Clin Exp Dermatol 2006;31:714–5. [9] Osio A, Fraitag S, Hadj-Rabia S, Bodemer C, de Prost Y, Hamel-Teillac D. Clinical spectrum of tufted angiomas in childhood: a report of 13 cases and a review of the literature. Arch Dermatol 2010;146:758–63. [10] Ferrandiz-Pulido C, Mollet J, Sabado C, Ferrer B, Garcia-Patos V. Tufted angioma associated with Kasabach–Merritt phenomenon: a therapeutic challenge. Acta Derm Venereol 2010;90:535–7.