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Discussion SCACP is one of the cutaneous adnexal neoplasm, and is a rare neoplasm showing apocrine differentiation [3]. The apocrine histiogenesis is supported by decapitation of the luminal surface of tall columnar cells, continuity of the tumour to pilo-sebaceous units and presence of apocrine glands in the underlying tissue [3]. It is considered to be a malignant counterpart of the SCAAP. Clinically, the long standing lesion suddenly begins to enlarge in size with bleeding, crusting and ulceration. It is most commonly seen in the head and neck region of elderly individual with no gender predilection [1,3]. Morphologically, SCACP resembles SCAAP with cystic papillomatous invaginations connected to the skin surface by funnel shaped structures lined by infundibular epithelium. The upper part of the cystic invaginations are lined by keratinizing squamous epithelium while the lower part and papillary projections are lined by inner columnar cells with decapitation and outer cuboidal cells. This epithelial transition from keratinizing squamous epithelium to glandular lining recapitulates the physiologic relationship of the apocrine gland to the hair follicle. In healthy skin, apocrine gland arises at the follicular infundibulum, characterized by a gradual transition from stratified squamous epithelium at the skin surface to the bi-layered ductal structures in the dermis [1,3]. Stroma of the tumour contains a dense inflammatory infiltrate of plasma cells and lymphocytes mirroring the attraction of plasma cells by glands of the normal secretory immune system [5]. SCACP differs from SCAAP and SCAAP IN SITU by infiltration of tumour cells into deep dermis or subcutaneous fat. SCACP and SCAAP IN SITU differ from SCAAP by cytological features of tumour cells characterized by higher nuclear cytoplasmic ratio, nuclear irregularity, coarse chromatin and increased mitotic activity [1,3,6]. Though there is no definitive immunohistochemical profile for a SCACP, the strong expression of CK7, CEA and GCFDP-15 supports the apocrine differentiation of the neoplasm [1]. It is well established that SCAAP shows positivity to CEA, CK7 and epithelial membrane antigen (EMA) in the luminal cells. However only 2 out of the 4 SCACP’s on which GCFDP-15 expression was evaluated, showed positive expression. Though there is not many published literature on the immunohistochemical characterization of SCACP, it is assumed that SCACP mirrors the immunohistochemical profile of SCAAP [7]. Our case was identified as SCACP since it showed the characteristic histology along with infiltration into deep dermis and presence of malignant cells. Also, the tumour strongly expressed apocrine differentiation markers CK7, CEA and GCFDP-15. Though a rare neoplasm, the entity should be recognized correctly as it may affect patient treatment and prognosis. Similar to other rare adnexal tumours, the literature reveals a good prognosis for SCACP patients treated only by surgical excision [8-10]. Acknowledgement Department of Histopathology, SRL Mumbai for performing the immunohistochemical characterization of the tumour. REFERENCES 1. Leeborg N, Thompson M, Rossmiller S, Gross N, White C, Gatter K: Diagnostic Pitfalls in Syringocystadenocarcinoma Papilliferum: Case Report and Review of the Literature. Archives of Pathology & Laboratory Medicine. 2010;134:1205-9. 2.Numata M, Hosoe S, Itoh N, Munakata Y, Hayashi S, Maruyama Y: Syringadenocarcinoma papilliferum. J Cutan Pathol. 1985;12:3- 7. 3. Park SH, Shin YM, Shin DH, Choi JS, Kim KH: Syringocystadenocarcinoma Papilliferum: A Case Report. J Korean Med Sci. 2007;22:762-5. 4. Abrari A, Mukherjee U: Syringocystadenocarcinoma papilliferum at unusual site: inherent lesional histologic polymorphism is the pathognomon. BMJ Case Reports. 2011;11:4254. 5. LeBoit PE, Burg G, Weedon D, Sarasin A, editors. Pathology and Genetics of Skin Tumours. Lyon: IARC; 2006. 6. Lindboe C, Brekke H, Schonhardt I, Houge U: Syringocystadenocarcinoma Papilliferum In Situ: Case Report with Immunohistochemical Observations. Scholarly Research Exchange. 2009;2009:1-4. 7. Ishida-Yamamoto A, Sato K, Wada T, Takahashi H, Iizuka H: Syringocystadenocarcinoma papilliferum: Case report and immunohistochemical comparison with its benign counterpart. J Am Acad Dermatol. 2001;45:755-9. 8. Dissanayake RVP, Salm R. Sweat-gland carcinomas: prognosis related to histological type. Histopathology. 1980;4:445-66. 9. Santa Cruz DJ: Sweat gland carcinomas: a comprehensive review. Semin Diagn Pathol. 1987;4:38-74. 10. Mlika M, Chelly B, Boudaya S, Ayadi-kaddour A, Kilani T, El Mezni F: Poroid hidradenoma: a case report. Our Dermatol Online. 2012;3:43-5. Copyright by Chidambharam Choccalingam, et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. © Our Dermatol Online 2.2013 223
Case Report DOI: 10.7241/ourd.20132.55 A COLUMELLAR DEFORMITY CAUSED BY A CONGENITAL SCHWANNOMA Mohamed El Bouihi, Saad Lahmiti, Souad Aimadeddine, Ahmed Zaroual, Saad Fawzi, Nadia Mansouri Hattab Source of Support: Nil Competing Interests: None Oral Maxilla-Facial and Aesthetic Surgery Department, Mohammed VI University Hospital, Marrakech School of Medicine, Morocco Corresponding author: Prof. Nadia Mansouri Hattab cmf.marrakech@gmail.com Our Dermatol Online. 2013; 4(2): 224-225 Date of submission: 06.01.2013 / acceptance: 09.02.2013 Abstract Schwannoma is a benign neoplasm arising from Schwann cells of the peripheral nerve. It very rarely located in the nasal tip. We report two cases of congenital schwannoma of the nasal columella and discuss the surgical approach of such tumor. Case report: Case 1: An 18 year-old female was referred to us for a very slow growing tumor of the columella. The deformity had been present since the birth. The patient underwent an excision of its tumor using an open rhinoplasty approach. The histological examination revealed a schwannoma. No recurrence was found within 2 years of follow up. Case 2: A 4 month male baby presenting a congenital tumor of his columella. He underwent an excision using open rhinoplasty approach. The histological examination showed a plexiform schwannoma. Discussion: Schwannoma of the nasal tip is a benign tumor that gradually causes aesthetic and functional disorders. Congenital schwannoma of columella is an extremely rare clinical situation. Its diagnosis and treatment can pose certain challenges. The treatment is surgical excision and histological analysis of the specimen. Open rhinoplasty approach provided a good surgical exploration and a good cosmetic result on this nasal tip tumor. Key words: nose neoplasms; neurilemmoma; rhinoplasty Cite this article: Mohamed El Bouihi, Saad Lahmiti, Souad Aimadeddine, Ahmed Zaroual, Saad Fawzi, N. Mansouri Hattab: A columellar deformity caused by a congenital schwannoma. Our Dermatol Online. 2013; 4(2): 224-225. Introduction Schwannoma is very rare in the nasal tip [1] and to the best of our knowledge congenital schwannoma of the nasal columella has never been reported. The diagnosis of such rare tumor is challenging and even if it is a benign neoplasm excision biopsy is essential to exclude sinister pathology [2]. We report a case of columellar schannoma. Case Report Case 1: A 18 year-old female, came to our service for a slow growing deformity of her columellar. The deformity had been present from the birth (Fig. 1). Physical examination revealed a well-defined elastic tumor in of the anterior part of the columella measuring 1cm. The overlying skin was thin. The patient underwent an excision of its tumor using an open rhinoplasty approach and a “V” shaped incision (Fig. 2). A superficial subcutaneous undermining allowed tumor removal. The medial crus of greater alar cartilage were slightly distorted. No nerve of origin was seen during the disssection. The limits of the excision were free of tumor, and histologic analysis of the tumor showed the characteristics of a schwannoma. The patient remains having no recurrence within 2 years of postoperative follow up. Case 2: A 4 month-old baby was referred to us for a congenital rapidly growing tumor of the columella (Fig. 3). On examination he presents an oval firm, mobile, globular swelling of the columella measuring 15 mm by 5 mm. The tumor had extended to the upper part of the philtrum and had caused a partial obstruction of the right nostril. Excision of the tumor was performed using an open rhinoplasty approach. Pathological examination revealed the diagnosis of plexifom schwannoma. Discussion Developmental midline nasal masses in children are rare, with a reported annual incidence of one in every 20,000–40,000 live births [2]. They result from a failure of embryologic separation of neuroectodermal and ectodermal tissues during the development of the nose and frontobasal region. Many such lesions may include an intracranial extension or connection. Their differential diagnosis includes abscesses, hemangiomas, fibromas, lipomas, granulomas, and mucoceles. 224 © Our Dermatol Online 2.2013 www.odermatol.com
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Volume 4, Number 2, April 2013 ISSN
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Editorial Board: Abdel-Naser Mohame
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CONTENTS E d i t o r i a l O r i g
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Original Article DOI: 10.7241/ourd.
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Figure 1. Survival time of HIV/TB-
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Figure 3. A. melanocytic naevus on
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82% of the children belonged to the
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Comment to the article DOI: 10.7241
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The skin around body openings such
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Figure 1. Finger nail dystrophy in
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Comment to the article DOI: 10.7241
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Similar type of rash was also prese
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13. Mastaglia FL, Ojeda VJ: Inflamm
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Page 34 and 35: 5. Brocard A, Quereux G, Moyse D, D
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Page 46 and 47: Discussion Poikiloderma of Civatte
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Page 52 and 53: Case Report A 56 year old Caucasian
Page 54 and 55: Using a battery of neurotoxic agent
Page 56 and 57: Figure 1. Multiple lesions on the u
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Page 62 and 63: The patient was put on melanocyl 0.
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Page 72 and 73: Figure 1. Histological section show
Page 76 and 77: Discussion The breast location of t
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Page 90 and 91: Clasificación En base al epitelio
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Page 100 and 101: Letter to the Editor DOI: 10.7241/o
Page 102 and 103: Historical Article DOI: 10.7241/our
Page 104 and 105: Figure 6. Henri Mondor (1885-1962).
Page 108 and 109: Figure 5. Alfred Guido Miescher (18
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Page 116 and 117: THOMAS INMAN English physician, 182
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