Malignant cylindroma in a patient with Brooke-Spiegler syndrome

Borik, Liliane; Heller, Patricia; Shrivastava, Monica; Kazlouskaya, Viktoryia

doi:10.5826/dpc.0502a09

Cited by 9 publications

(2 citation statements)

References 22 publications

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“…The tendency of malignancy is higher in multiple cylindromas than in the solitary type. Only less than 50 malignant cylindroma cases are reported [ 10 , 13 ]. Clinical signs that suggest malignant transformation are sudden and rapid tumor growth, necrosis, ulceration, blue-to-pinkish discoloration, and hemorrhage [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Solitary Dermal Cylindroma in an Uncommon Site: A Rare Case Report

V.¹,

Thanka²,

Srismitha³

et al. 2022

Cureus

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Cylindroma, a benign skin appendage tumor, mostly occurs in the head and neck region. They present as pink to purple, smooth-surfaced, solitary or multiple nodular lesions, mostly affecting middle-aged and elderly women. A clinical diagnosis of cylindroma is unusual but must be considered clinically. We report an unusual case of solitary benign dermal cylindroma that occurred in an uncommon site in a 54-year-old female. She came with complaints of painful swelling on the left side of her chest wall for six months. Histopathological examination of the mass revealed a well-defined tumor composed of nests and islands of a dual population of tumor cells surrounded by pink basement membrane-like material. These nests of cells are arranged in a "jigsaw" puzzle fashion, which is typical of cutaneous cylindroma. The thick pink material surrounding the epithelial cell islands is periodic acid Schiff stain (PAS) positive.

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Section: Discussionmentioning

confidence: 99%

Solitary Dermal Cylindroma in an Uncommon Site: A Rare Case Report

V.¹,

Thanka²,

Srismitha³

et al. 2022

Cureus

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“…Most frequently, neoplasms resemble salivary gland basal cell adenocarcinomas. The coexistence of both benign and malignant variants of salivary gland tumors with cylindromas/cylindrocarcinomas has to be kept in mind 7 . The other way round, 10.8% of basal cell adenocarcinomas of parotid and submaxillary glands reveal other neoplasms of skin adnexal type, including dermal cylindroma, eccrine spiradenoma and trichoepithelioma 8 …”

Section: Discussionmentioning

confidence: 99%

Surgical Treatment of Brooke-Spiegler Syndrome

Engels¹,

Fritsche²,

Pabst

et al. 2020

Journal of Craniofacial Surgery

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confirmed. The patient's vision loss and eye movements improved after the operation (Fig. 1C). In the examination, the light reflex was completely natural. DISCUSSIONOnodi cell, which is a type of ethmoid cells, is seen in routine radiological imaging at 8% to 14% and in cadaver studies up to 60%. 8,9 Its close proximity to the orbital apex increases the clinical importance of the lesions of this area. Onodi cell mucocele has been shown extremely rarely in the literature. Mucocele actually arises with decreased sinus drainage. It is a slow growing, mucus containing cystic lesion in benign nature. Clinically, it is generally asymptomatic but it may present with various compression findings depending on the location. 6,10 Direct mass compression effect was shown primarily in the pathogenesis of optic neuropathy for onodi cell mucocele. Apart from this, superinfection and ischemia, which develop as a result of compression of the vasa nervorum, are among other causes. Response to treatment is good even in patients without infection and inflammation.Radiological imaging methods play an important role in the diagnosis of mucocele, since the lesion has no specific clinical and laboratory findings. This importance increases even more in patients presenting with compression symptoms. With imaging, the diagnosis of mucocele is easily made and it is a guide for the necessary surgical procedure. The onodi cell mucocele shows the cystic enlargement structure typically found in the upper lateral of the sphenoid sinus. It can be monitored at different intensity and density due to the amount of protein in the mucus content and secondary infections. Although post-contrast imaging shows peripheral minimal enhancement, it is not usually contrasted. While CT is good at showing the expansion in the bony structure, MR clearly reveals the relationship with neighboring structures. The treatment is surgical resection with an endoscopic approach. 7,11 In conclusion, as we have shown in our case, onodi cell mucocele is a rare cause of sudden vision loss. Imaging methods are essential for early diagnosis and promptly treatment, so patients' symptoms can be reversible.

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