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Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 439-441
Catechism (Quiz 12)

1 Department of Surgical Pathology, Tata Memorial Hospital (TMH), HBNI University, Parel, Mumbai, Maharashtra, India
2 Department of Surgical Oncology (Bone and Soft Tissues), Tata Memorial Hospital (TMH), HBNI University, Parel, Mumbai, Maharashtra, India

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Date of Submission14-Jan-2021
Date of Acceptance10-Feb-2021
Date of Web Publication9-Apr-2021

How to cite this article:
Rekhi B, Bal M, Gulia A. Catechism (Quiz 12). Indian J Pathol Microbiol 2021;64:439-41

How to cite this URL:
Rekhi B, Bal M, Gulia A. Catechism (Quiz 12). Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 9];64:439-41. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/439/313290

A 69-year-old male presented with an ulcer toward the left side over his tongue of 3 months duration. He disclosed a history of hypertension and diabetes mellitus (Type II). He underwent total laryngectomy and postoperative radiation therapy 30 years ago.

During his recent clinical examination, a 3 cm × 2 cm sized lesion was noted over the left lateral border his tongue. He underwent positron emission tomography and computed tomography (PET-CT), which revealed an increased tracer uptake, because of an enhancing lesion involving the left lateral border of his tongue. This was diagnosed as a moderately differentiated squamous cell carcinoma.
Figure 1:

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Figure 2:

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In addition, a fluorodeoxyglucose (FDG)-avid mass was noted in his left pectineus and adductor longus, measuring 2.7 cm × 2.3 cm, with SUV max = 14.4.

Magnetic resonance imaging (MRI) revealed a well-defined soft tissue lesion measuring 2.7 cm × 2.5 cm × 1.5 cm in the intermuscular plane in the medial compartment of the proximal left thigh, involving the pectineus and adductor longus muscles. The lesion was hypointense on T1, heterogeneous on T2-weighed images, including hypointense areas, and displayed post-contrast enhancement. It was seen focally abutting the superior pubic ramus.

Images from the initial biopsy and subsequent excision of the lesion in his pectineus are provided.


Q1: What is the diagnosis?

Q2: What are the main subtypes of this tumor?

Q3: Which additional tests can be useful in such a case?

Q4: What is the characteristic underlying molecular feature associated with this tumor?

   Answers of Catechism (Quiz 11) Top

The histopathological diagnosis is nevus sebaceus (NS) of Jadassohn, also called as organoid nevus or pilosyringosebaceous nevus. It is an example of a rare cutaneous hamartoma (incidence of less than 1/100,000).[1] It is thought to be caused by post-zygotic mosaic mutations in the RAS family of oncogenes (the group of mosaic RASopathies) and some of the cases are associated with cerebral, ocular, and skeletal defects (nevus sebaceous syndrome).[2] NS is most commonly located on the scalp followed by face, where the lesion starts as a hairless plaque or linear streak. At puberty, there is an enlargement (most likely induced by sex hormones) with a verrucous or nodular appearance.[1]

NS, despite its name, is a hamartoma of the pilosebaceous and follicular unit and therefore shows a divergent differentiation.[1] The sebaceous glands are immature [Figure 1a, arrow] and may also show hyperplasia and crowding. The hair follicles are abortive in the form of cords of undifferentiated cells resembling the embryonic stage [Figure 1b, black arrow], which mimics basal cell carcinoma. Epidermal changes are seen in the form of acanthosis, papillomatosis, hyperkeratosis and dilated, keratin-filled infundibula [Figure 1b, white arrow]. Dilated eccrine glands and prominent apocrine glands may also be present. The histolopathology of the lesion varies with age, which may lead at times to a misdiagnosis.[1],[3] In the infantile stage, the epidermis is only slightly acanthotic and hyperpigmented while the hair follicles are small and incompletely formed, in the form of solid cords of undifferentiated basaloid cells. The sebaceous glands are not prominent. In the adolescent stage, verrucous epidermal hyperplasia is prominent. In the adult stage, epidermal hyperplasia, large sebaceous glands, and ectopic apocrine glands are predominant while the hair follicles remain primordial.

The most definitive treatment of nevus sebaceous is excision, which is performed not only for cosmetic reasons but also for prevention of benign and malignant tumors that arise from NS.[1] The risk of malignancy is 0.9–2.5%. The commonest tumors are trichoblastoma, syringocystadenoma papilliferum and basal cell carcinoma; simultaneous occurrence of multiple tumors is also reported. However, the necessity and timing of excisions are not well established. Since the risk of malignancy is not too high, lesions that are not disfiguring may be clinically followed up without excision.[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Kelati A, Baybay H, Gallouj S, Mernissi FZ. Dermoscopic analysis of nevus sebaceus of Jadassohn: A study of 13 cases. Skin Appendage Disord 2017;3:83-91.  Back to cited text no. 1
Simi CM, Rajalakshmi T, Correa M. Clinicopathologic analysis of 21 cases of nevus sebaceus: Clinicopathologic analysis of 21 cases of nevus sebaceus: A retrospective study. Indian J Dermatol Venereol Leprol 2008;74: 625-27.  Back to cited text no. 2
[PUBMED]  [Full text]  
Chun SH, Kim BY, Park JH, Kim I, Ryu HJ. Simultaneous presentation of trichilemmal carcinoma and syringocystadenoma papilliferum within a nevus sebaceous. Ann Dermat 2018;30:368-70.  Back to cited text no. 3
Kamyab-Hesari K, Seirafi H, Jahan S, Aghazadeh N, Hejazi P, Azizpour AA. Nevus sebaceus: A clinicopathological study of 168 cases and review of the literature. Int J Dermatol 2016;55:193-200.  Back to cited text no. 4

Correspondence Address:
Bharat Rekhi
Room Number 818, Department of Surgical Pathology, 8th Floor, Annex Building, Tata Memorial Hospital, Dr E.B. Road, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_41_21

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