research article “troublesome tumors” of the skin ... · most tumors are found to arise de novo...

International Journal of Medical Science and Public Health 7142018 | Vol 7 | Issue 9

“Troublesome tumors” of the skin: Spectrum of skin adnexal tumors at a tertiary care center in Malwa region

Sarjana Agrawal, Ravi Jain, Ashok Panchonia, Kulkarni C V, Rakesh Mehar

Department of Pathology, Mahatma Gandhi Memorial Medical College, Indore, Madhya Pradesh, India

Correspondence to: Sarjana Agrawal, E-mail: [email protected]

Received: March 23, 2018; Accepted: January 01, 2018

INTRODUCTION

Skin adnexal tumors are morphologically diverse, rare group of neoplasms of unknown origin. Depending on their presumed origin, these tumors of the epidermal appendages have been traditionally categorized into those with apocrine and eccrine, follicular and sebaceous differentiation.[1] Quite interestingly, while a significant percentage of adnexal neoplasms manifests

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DOI: 10.5455/ijmsph.2018. 0308801062018

predominantly one line of differentiation; a morphologically overlapping picture not only among themselves but also with surface epidermal type[2] is often encountered in practice which can pose a diagnostic dilemma. Recent researches have postulated a multipotential stem cell origin of these tumors, present within the epidermis or its appendageal structures, rather than a mature, differentiated cell origin.[3] This explains the considerable plasticity as well as resemblance of these tumors with their mature counterparts. To some extent, the differentiation of neoplasms probably reflects their distribution.[1]

Most tumors are found to arise de novo or from a malformation involving the epidermis and adnexae.[1] Often, a diagnosis of exclusion, majority have a benign predisposition with similar morphology. Malignant skin adnexal neoplasms are even

Background: Skin adnexal tumors are uncommon in routine practice and usually cause diagnostic problems because of their rarity. Objective: The aim of this study was to find the prevalence of skin adnexal tumors among all the skin biopsies received at the Department of Pathology at M.Y. Hospital, Indore. Materials and Methods: The study was conducted prospectively and retrospectively and records and slides prepared between May 2014 and December 2017 at the Pathology Department, M.Y. Hospital were studied. All the skin biopsies received during this period was evaluated, and diagnosis was made on hematoxylin and eosin stained slides of the processed tissue received. The lesions were then characterized according to the WHO histological classification of appendageal tumors. Results: Of 560 skin biopsies received, during the 3.5 years period, only 47 (7.2%) cases were diagnosed as skin adnexal tumors on histopathology. 42 cases (89.36%) were found to be benign, while 5 cases (10.63%) were malignant. Pilar tumors (48.93%) were the most common tumors encountered, followed by eccrine/apocrine tumors (29.79%) and sebaceous tumors (21.28%). Tumors were observed more commonly in females with 21–40 years being the predominant age group. Most tumors were localized to head and neck region (57.4%), followed by trunk and upper limb (17.02%), abdomen and lower limb (17.02%), and inguinal region (8.5%). Conclusion: The overall prevalence of skin adnexal tumors was found to be very low with a benign morphology in most cases. Often, clinically misdiagnosed due to rarity of occurrence; a thorough histopathological assessment is mandatory for accurate diagnosis.

KEY WORDS: Adnexal Tumors; Pilar Tumor; Eccrine Tumors; Apocrine Tumor; Sebaceous Tumor

ABSTRACT

International Journal of Medical Science and Public Health Online 2018. © 2018 Sarjana Agrawal, et al. This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material for any purpose, even commercially, provided the original work is properly cited and states its license.

Research Article

Agrawal et al. Spectrum of skin adnexal tumors

715 InternationalJournalofMedicalScienceandPublicHealth 2018 | Vol 7 | Issue 9

rarer in incidence with a tendency of recurrence and may metastasize to skin or lymph nodes.[4]

A clinical misdiagnosis is quite rampant owing to their rarity of occurrence with uncommon demographics, anatomic location and distribution often confounding the picture. A meticulous histomorphological examination is thus an absolute must in all cases.

A significant clinical association with internal visceral malignancies has been documented with a considerable number of these tumors, which again justifies the need to study these troublesome tumors in depth beyond just academic curiosity.

The present study was conducted to determine the prevalence of the skin adnexal tumors in the hospital-based population in Malwa region. The frequency distribution of tumors on the basis of their line of differentiation on histological grounds and their location was determined. The age- and sex-specific incidence of the histological types was also evaluated.

MATERIALS AND METHODS

The study was conducted over a 3.5 years period, both prospectively and retrospectively.

All the skin biopsies received during this period were evaluated, and diagnosis was made on hematoxylin and eosin stained slides of the processed tissue received. The records and slides prepared of the lesions before May 2015 were also considered in the study. The lesions were then characterized according to the WHO histological classification of appendageal tumors.

RESULTS

The study included 47 cases of all age groups ranging from 9 to 90 years. The mean age of presentation was 43.8 years. The tumors were found to be slightly more prevalent among females with an M: F ratio of 1:1.47. Of the 47 cases studied, only 5 cases were found to be malignant, while the rest of them were benign. On the basis of their adnexal differentiation, the most frequent group of tumors were of follicular origin (48.93%) followed by eccrine/apocrine origin (29.79%) and sebaceous origin (21.28%) [Table 1].

Pilomatrixoma (19.13%) was the most common tumor encountered in the study followed by pilar cyst (14.89%). Both these tumors belong to tumors with follicular differentiation. Next, in frequency was nodular hidradenoma (10.63%) which belongs to eccrine group of appendageal tumors. Sebaceous adenoma (8.51%) was the predominant benign sebaceous tumors studied, whereas sebaceous carcinoma and basal cell epithelioma with sebaceous differentiation (4.26%) were

among the majority of rare malignant skin adnexal tumors seen in the study. A malignant skin adnexal tumor with predominant eccrine differentiation was also included among the cases [Table 2].

Most tumors of the follicular and eccrine group were observed in the 21–40 years age group while the sebaceous

Table 1: Frequency of tumors on the basis of differentiation in the study

Tumor differentiation Frequency (%)Follicular 23 (48.93)Eccrine/apocrine 14 (29.79)Sebaceous 10 (21.28)Total 47 (100)

Table 2: The frequency distribution of the various adnexal skin tumors

Tumor group on the basis of origin

Frequency % of subtypes

% of total SAT

Follicular tumors (F)Pilomatrixoma 9 39.16 19.13Trichoepithelioma 4 17.34 8.51Trichoadenoma 2 8.69 4.26Trichofolliculoma 1 4.35 2.13Pilar cyst 7 30.46 14.89Total 23 100.00

Eccrine/apocrine tumors (E)

Nodular hidradenoma 5 35.72 10.63Hidradenoma papilliferum

3 21.44 6.38

Syringoma 2 14.28 4.26Cylindroma 2 14.28 4.26Eccrine acrospiroma

1 7.14 2.13

Total 13 92.86Sebaceous tumors (S)

Sebaceous adenoma 4 40 8.51Sebaceous carcinoma 2 20 4.26Nevus sebaceous 2 20 4.26Total 8 80

Unclassified/more than one origin

Basal cell epithelioma with sebaceous differentiation (S)

2/10 20 4.26

Skin adnexal tumor with predominant eccrine differentiation (E)

1/14 7.14 2.13

Total 47 100.00



tumors were seen in the 41–80 years bracket [Table 3].

Head and neck (57.44%) was the most common site involved in both males and females with a predominance in the facial region [Table 4].

Pilomatrixoma (19.13%) [Figure 1] was the most common tumour encountered in the study followed by pilar cyst (14.89%) [Figure 2]. Both these tumours belong to tumours with follicular differentiation. Next in frequency were nodular hidradenoma (10.63%) [Figure 3] which belong to eccrine group of appendageal tumours. Sebaceous adenoma (8.51%) were the predominant benign sebaceous tumours studied whereas sebaceous carcinoma [Figure 6] and basal cell epithelioma with sebaceous differentiation (4.26%) [Figure 5] were among the majority of rare malignant skin adnexal tumours seen in the study. A malignant skin adnexal tumour with predominant eccrine differentiation [Figure 4] was also included among the cases.

DISCUSSION

Skin adnexal tumors are morphologically diverse, rare group of neoplasms; mostly benign in nature as confirmed by this study. With an 8.3% (47/560) prevalence in the hospital-based population, over a 3.5-year study period; only 5/47 (10.64%) tumors were found to be malignant. All the malignant cases were seen in the 4th–6th decade in the females. Overall, the incidence was skewed toward the female gender with predominant cases in the 21–40 years age group. On the basis of line of differentiation, follicular group of tumors was in majority with eccrine and sebaceous tumors next in

frequency. While most follicular and eccrine tumors were found to be benign, sebaceous group of tumors formed the majority of malignant tumors encountered in the study. The head and neck region was found to be the most preferred location for these tumors, in accordance with the standard literature, which rather defers from postulating a sun exposure as a definitive triggering event in the majority of these tumors. The extremities and inguinal region were also found to harbor these tumors in significant frequency in the study.

In this study, the tumor prevalence was studied among 9–90 years age group. The mean age of presentation was 43.8 years. Several other studies have reported a marked variation among the age of presentation of these tumors. While Saha et al.[5] observed the mean age of onset of the skin adnexal tumors as 24.15 ± 8.44; Vani et al.,[6] Sharma et al.,[7] and Alam et al.[8] found the predominant prevalence in the 4th–5th decade in their study, and Radhika et al.[9] had majority of their cases in the 3rd decade.

It is important to note that while the benign tumors had a much earlier age of presentation; the malignant tumors presented most often in the elderly. The relative predominance of the benign lesions with a rare incidence of these tumors can account for the skewed age distribution in various studies.

The present study had 89.36% cases reported as benign in nature, in agreement with the other studies by Vani et al.,[6] Sharma et al.,[7] and Alam et al.[8] who reported a 74.5%, 80.36%, and 92.3% benign appendageal tumors in their study.

On the basis of the line of differentiation, this study found a majority of follicular tumors followed by eccrine and sebaceous tumors. Pilomatrixoma was found as the most common tumor among all adnexal tumors studied. Other studies by Vani et al.,[6] Sharma et al.,[7] and Radhika et al.[9] have reported a majority of eccrine tumors with nodular hidradenoma as the most common tumor reported in their study. Alam et al.[8] have reported sebaceous tumors as the most common tumors in their study with sebaceous hyperplasia and sebaceous carcinoma as the most common tumors encountered by them.

While follicular and eccrine tumors were most prevalent in the 21–40 years age group, the sebaceous tumors were mostly found in the 4th–6th decade in this study. Other studies report a

Table 3: Age incidence of adnexal tumors in the studyAge group Follicular (%) Eccrine/apocrine (%) Sebaceous (%) Total (%)0–20 4 (17.4) 1 (7.1) 2 (20) 7 (14.89)21–40 10 (43.5) 5 (35.7) 2 (20) 17 (36.17)41–60 7 (30.4) 4 (28.5) 3 (30) 14 (29.79)61–80 2 (8.7) 3 (21.4) 3 (30) 8 (17.02)>80 0 1 (7.1) 0 1 (2.13)Total 23 14 10 47 (100.00)

Table 4: Site and sex distribution of cases studiedSite of tumor Male Female Total % AgeHead and Neck

Scalp 3 2 5 10.64Face 7 12 19 40.42Neck 1 2 3 6.38

Total 27 57.44Trunk and upper limb 2 6 8 17.02Abdomen and lower limb 6 2 8 17.02Inguinal region 0 4 4 8.52Total 19 28 47 100.00


717 InternationalJournalofMedicalScienceandPublicHealth 2018 | Vol 7 | Issue 9

slightly higher age at presentation as compared to this study. The geographical factors and awareness among subjects may be a contributing cause.

The tumors were found to be slightly more prevalent among the females with an M: F ratio of 1:1.47. This is in accordance with the other studies as well as the standard literature.

The distribution of the tumors was found most frequently in the head and neck region (57.4%) with a predisposition in the facial region in both the genders. The trunk and extremities were the next common regions affected

Figure 1: Pilomatrixoma (×40, H and E)

Figure 2: Pilar cyst (scanner view, H and E)

Figure 3: Nodular hidradenoma (×40, H and E)

Figure 4: Malignant skin adnexal tumor with predominant eccrine differentiation (scanner view, H and E)

Figure 5: Basal cell epithelioma with sebaceous

Figure 6: Sebaceous carcinoma (scanner view, H and E) differentiation (scanner view, H and E)



followed by the inguinal region. While the male population showed a higher distribution in the upper extremities, the female population showed a predisposition to the lower extremities.

The study thus comprehensively studied the histopathology of the appendageal tumors with respect to demography and anatomic location in a hospital-based population in the Malwa region. Special stains and immunoprofile of the tumors were not studied in the cases. The standard literature describes a complex array of cytokeratins, carcinoembryonic antigen, and epithelial membrane antigen[1] to be assessed in cases with more than one line of differentiation. While the tumors encountered were mostly benign, their malignant counterparts pose a challenge to discover if the tumors evolve as a continuum or with malignancy as the first hit in their development sequelae.

CONCLUSION

The overall prevalence of skin adnexal tumors is very low among the overall skin lesions encountered. Histopathology provides an efficient diagnostic tool to classify the tumors on the basis of their line of differentiation as well as a benign or malignant nature. Most tumors were found to be benign with follicular tumors as the majority of cases in the study. The age presentation was in the older decades for malignant tumors while the benign tumors presented in 21–40 years age group, with a slightly female predominance in both groups. The predominant site affected was the head and neck region which may account for their early detection and management.

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1. Le Boit PE, Burg G, Weedon D, Sarasin A. World Health Organization classification of Tumours. Pathology and

Genetics of Skin Tumors. 3rd ed., Vol. 6. Lyon: IARC Press; 2005. p. 122-4.

2. Wong TY, Suster S, Cheek RF, Mihm MC. Benign cutaneous adnexal tumors with combined folliculosebaceous, apocrine, and eccrine differentiation: Clinicopathologic and immunohistochemical study of eight cases. Am J Dermatopathol 1996;18:124-36.

3. Perez-Losada J, Balmain A. Stem-cell hierarchy in skin cancer. Nat Rev Cancer 2003;3:434-43.

4. Klein W, Chan E, Seykora JT. Tumors of Epidermal Appendages. Lever’s Histopathology of the Skin. 9th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2005. p. 867-926.

5. Saha A, Das NK, Gharami RC, Chowdhury SN, Datta PK. A clinicohistopathological study of appendageal skin tumors, affecting head and neck region in patients attending the dermatology OPD of a tertiary care centre in Eastern India. Indian J Dermatol 2011;56:33-6.

6. Vani D, Ashwini N, Sandhya M, Dayananda TR, Bharthi M. A 5 year histopathological study of skin adnexal tumors at a tertiary care hospital. IOSR J Dent Med Sci (IOSR-JDMS) 2015;14:1-5.

7. Sharma A, Paricharak DG, Nigam JS, Rewri S, Soni PB, Omhare A, et al. Histopathological study of skin adnexal tumours-institutional study in South India. J Skin Cancer 2014;2014. Article ID: 543756.

8. Alam S, Lateefa M, Mohanty R. Histopathological study of 26 rare skin adnexal tumours over 5 years–a diagnostic dilemma! Int J Med Sci Public Health 2016;5:1995-8.

9. Radhika K, Phaneendra BV, Rukmangadha N, Reddy MK. A study of biopsy confirmed skin adnexal tumors: Experience at a tertiary care teaching hospital. J Clin Sci Res 2013;2:132-8.

How to cite this article: Agrawal S, Jain R, Panchonia A, Kulkarni CV, Mehar R. “Troublesome tumors” of the skin: Spectrum of skin adnexal tumors at a tertiary care center in Malwa region. Int J Med Sci Public Health 2018;7(9):714-718.

Source of Support: Nil, Conflict of Interest: None declared.

research article “troublesome tumors” of the skin ... · most tumors are found to arise de novo...

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