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\begin{document}

\title{Recurrent facial Microcystic adnexal carcinoma and hair transplantation
on scar site}



\author[1]{amir mohammad beyzaee}%
\author[2]{Mohamad Goldust}%
\author[3]{Anant Patil}%
\author[1]{Ghasem Rahmatpour Rokni}%
\author[1]{Bahare Ghoreishi }%
\affil[1]{Mazandaran University of Medical Sciences}%
\affil[2]{Johannes Gutenberg Universitat Mainz}%
\affil[3]{Padmashree Dr DY Patil Medical College}%


\vspace{-1em}



  
  \date{May 26, 2024}


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\textbf{Recurrent facial Microcystic adnexal carcinoma and hair
transplantation on scar site}

\textbf{Abstract}

\textbf{Introduction} : Microcystic adnexal carcinoma (MAC) is a rare
kind of cutaneous neoplasm with a very aggressive local infiltration
that destructs the affected tissues. Its rate of recurrence is high and
it mostly involves the face and scalp regions and most of the patients
get affected in the fourth or fifth decades of their life.

\textbf{Case presentation:} Here in, we report a 61-year-old woman with
a right-sided eyebrow MAC lesion with recurrency.

\textbf{Result:} Total excisional surgery was performed. A-T Flap
surgery was applied on the involved area, and after a 2-year period of
follow up, with no recurrency, hair transplantation with Follicular Unit
Transplantation method was successfully performed on the scarred area.

\textbf{Conclusion:} Although microcystic adnexal carcinoma is an
uncommon neoplasm; dermatologists and ophthalmologists should consider
it as a differential diagnosis, due to its aggressive local
infiltration. Complete surgical excision and long-term follow up must be
applied to manage the disease. Also, hair transplantation with
follicular unit transplantation technique can be considered as a
beneficial method for treating scars resulted from MAC excisional
surgery.

\textbf{Key words:} Microcystic adnexal carcinoma, facial tumor, hair
transplantation, scar



\textbf{Introduction}

Microcystic adnexal carcinoma (MAC), firstly described by Goldstein in
1982 (1), is a rare kind of cutaneous neoplasm with a very aggressive
local infiltration that destructs the affected tissues (2). MAC recurs
frequently and the ratio of male and female patients affected by, is
almost equal. Head and neck are the most involved regions.

Its age range is 11 to 83 years (mostly appears in the 40 to 50) and is
described as a firm, colored lesion (papule, nodule, or plaque) which
grows slowly (2-6).

Pain, ignition, and decreased sensation or paresthesia (which is a sign
of perineural invasion) are considered as the most common symptoms of
MAC (2,7-10).

Although MAC is not a metastatic tumor (5); it infiltrates, shelvs, or
skates among the facial tissues (including muscle, perichondrium,
periosteum, and galea) and aggressively invades the affected region (8)
and spreads far beyond the visible margins (11).

According to its clinical and histological features, it can easily be
misdiagnosed or confused with other cutaneous neoplasms like basal cell
carcinoma, squamous cell carcinoma, desmoplastic trichoepithelioma,
syringoma, and trichoadenoma (12).

The correct diagnosis of MAC is very vital because of its aggressive
behaviour and high recurrence rate. Here in, we report a case of
recurrent MAC and discuss about possible treatments.

\textbf{Case presentation}

A 61-year-old woman referred to our dermatology clinic with a recurrent
lesion on her right eyebrow since the last 3 years. The lesion had no
pain and had been enlarging slowly during these years. She declared
avoidance of excessive sun exposure or radiotherapy on the head or neck
region. Excisional surgury had been performed twice on the lesion, and
it has been recurred again now.

On the examination, there was a round and elevated lesion with 25 * 30
mm diameter on the right eyebrow. The surface of the lesion was nodular
and indurated (Figure 1). The lesion was firm and had no attachment to
the underlying periosteum; no skin ulceration or regional
lymphadenopathy was seen. The patient declared no tenderness.

After excision biopsy, pathology reported adnexal neoplasm consistent
with microcystic adnexal carcinoma.

Total excisional surgery was performed. Finally, A-T Flap surgery was
applied on the involved area (figure 2).

The physicians offered her to do radiotherapy, but she refused.

After a 2-year period of follow up, with no recurrency, hair
transplantation with Follicular Unit Transplantation method was
successfully performed on the scarred area (figure 3).

\textbf{Discussion}

MAC mostly occurs in the centrofacial region, in which lips include
about 50\% of cases (8).

The other most common involved sites are periocular area, nose,
nasolabial fold, cheek, scalp, and chin. Although MAC is mostly
considered as a neoplasm of the head and neck region; the axilla,
antecubital fossa, buttocks, foot, and chest are the other affected
areas which have been reported in the literature (2,4,12,13).

MAC is assumed to be a non-metastatic tumor; however, Yugueros et al
reported 55 patients affected to sweat gland carcinomas, in which, 17 of
them declared a history of MAC which was treated by excisional surgery
and radiotherapy (14). Also Lei et al. reported lymph node involvement
in the contralateral region of neck in a patient with MAC, which had
performed radiation and surgery as treatment 22 years earlier. They
proposed that it is more possible to be the result of direct extension
via perineural path rather than lymphatic spread (4,15). The
supraorbital nerve can be invaded by the lesions presenting in
periorbial area, particularly on the eyelid or eyebrow.

Although no defenitive cause has been found yet, some studies have
reported radiation therapy, chronic sun exposure, and immuno-compromised
status as predisposing factors for MAC (2,3,8,16).

History of prior radiation has been reported in 8--12\% of the cases
(17-19); also lesions in atypical sites, including the neck, have been
seen in patients with a history of radiation on the affected area
(20,21).

It is proposed that taking immuno-suppressive drugs may develop MAC
lesions (22). A chronic lymphocytic leukemia (CLL) patient with scalp
MAC was reported by Carroll et al. (23); as it has been reported in
immuno-compromised patients several times (22-24).

In order to have a successful treatment, an accurate diagnosis is
necessary. Clement et al. found that MAC can be easily misdiagnosed
(25). As mentioned before, the clinical presentation of MAC could be
mistaken for basal cell carcinoma (BCC), squamous cell carcinoma, cyst
or scar, particularly when a biopsy is not representative of the entire
lesion. Failure to obtain a proper biopsy sample could also result
misdiagnosis, due to the infiltrative nature of the lesion.

We can not evaluate the treatment options of MAC properly, due to the
rarity of the disease and lack of studies with long-term follow-up of
the patients in the literature. As recurrence of MAC is not predictable,
exision surgery with clear margins and minimum pathologic atypia can aid
to decrease the rate of recurrence (2).

The most common utilized treatment modalities for MAC include wide local
excisional surgery (WLE), Mohs micrographic surgery (MMS) and
radiotherapy. Recurrence has been reported in using every type of
treatment modalities above, even in patients with clear tumor margins
(3,8, 15,26).

Regardless of the method chosen, complete removal of the tumor on its
first occurrence is ideal as recurrent lesions are much more difficult
to manage.

MMS offers the highest probability of clear surgical margins as it can
detect subclinical extension while providing maximum tissue sparing
(2,27).

Determining true margin status in the subset of MAC patients with
perineural invasion (PNI) treated with surgical techniques can be
problematic. Cutaneous carcinoma with PNI manifestation, behaves
aggressively and increased rate of recurrence with wide extension have
been observed. It has been reported in 17.5--59\% of MAC patients (27).

WLE is another alternative, in which margin detection depends mainly on
the histologic technique hired by the pathologist.

It is strongly recommended to perform complete excisional surgery with
clear margins with long-term follow-up, because of high rate of
recurrences; as it can recur even several years after the surgery
(2,28). Also we should take surgical margins wider than clinical margins
due to the infiltrative growth pattern of the tumor. Recurrence rate of
WLE is about 17\% to 60\%, as reported in studies (2,8,29,30).

It is still challenging to define the role for definitive or adjunctive
radiotherapy in, because of the rarity of disease and the uncommon use
of radiotherapy for managing the disease. The majority of studies
reported unclear details about hired techniques of radiotherapy for MAC
lesions. Differences in radiotherapy techniques and sequencing also make
it difficult to draw conclusions. There is evidence that radiotherapy
can transform the MAC lesion into a new highly aggressive form,
according to its clinical and histological features (31,32).

MAC has also been reported as a radiogenic secondary malignancy (33). 16
of 84 published MAC cases in the literature, have declared a history of
prior exposure to radiations, with 19.05\% incidence
(8,17,19,28,31,34-44).

As the rate of MAC recurrence after monotherapy with radiotherapy is
high (1,8,45), it is not commonly suggested to use radiotherapy as
definitive monotherapy for patients with MAC. But as adjuvant therapy,
it may be useful in subset of patients who have undergone WLE or MMS
without achieving a tumor free margin and are at high risk of
recurrence. Also it can be considered as treatment in patients who avoid
to perform WLE or MMS, because of the disfiguring facial defects of
surgery. Elderly patients may also be a good candidate for radiotherapy,
particularly in those whose comorbidities make them poor surgical
candidates.

The knowledge about hair transplantation is growing every day. In 1959,
Dr. Orentreich developed it for the first time. He applied it as a
therapeutic procedure on a patient suffering from androgenic alopecia
(46).

Hair transplantation is used for covering the features of hair loss and
it has become one of the main popular procedures in cosmetic field
(particularly scars) now with satisfactory results (47).

Follicular unit transplantation (FUT) and follicular unit extraction
(FUE) are common techniques hired for hair transplantation. In FUT
technique, a band-shaped tissue of occipital region is used as donor,
which results a linear scar. To avoid its complications, including
scarring, FUE technique was introduced, in which, small units of hair
follicles are harvested (47).

Osman et al. performed hair transplantation on the cleft lip area scar.
They hired fat grafting procedure on twenty patients suffering from scar
and alopecia. Then hair transplantation was performed three months after
fat injection. After a one-year period of follow-up, patients declared
noticable high level of satisfaction (48).

Also Soyeon et al. evaluated the results of hair transplantation for
scar management in 25 cases (of 23 patients), in which burns, operation,
and trauma resulted scar with hair loss on the scalp and the face
(eyebrow, lip, and eyelid). After a 6 months period of follow-up,
satisfactory report from hair follicle transplantation procedure was as
excellent (44.4\%), good (38.9\%), fair (11.1\%), and poor (5.6\%) (49).

Transplanting the hair follicles on a tissue with scar is so difficult,
due to the poor blood supplement and tenacity of the scar tissue. Also,
a higher rate of success and favorable results is seen in patients with
burned scar rather than incision scars, due to the deeper depth of
incision scars (49).

So, we should always warn the patients that choosing hair follicle
transplantation procedure for scar management, can be accompanied by
secondary (or more) operations, for better cosmetic result.

\textbf{Conclusion}

Although microcystic adnexal carcinoma is not a common tumor;
dermatologists and ophthalmologists should consider it as a differential
diagnosis, due to its aggressive local infiltration. Complete surgical
excision and long-term follow up must be applied to manage the disease.

Also, hair transplantation with follicular unit transplantation
technique can be considered as a beneficial method for treating scars
resulted from MAC excisional surgery.

We declare that none of the authors have any conflict of interest.

\textbf{Reference} :

1. Goldstein D, Barr R, Santa Cruz D. Microcystic adnexal carcinoma: a
distinct clinicopathologic entity. \emph{Cancer} 1982;50:566--72.

2. Cooper PH, Mills SE, Leonard DD. Sclerosing sweat duct
(syringomatous) carcinoma. \emph{Am J Surg Pathol} 1985;9:422--33.

3. Friedman PM, Friedman RH, Jiang SB. Microcystic adnexal carcinoma:
collaborative series review and update. \emph{J Am Acad
Dermatol}1999;41:225--31.

4. Lei JY, Wang Y, Jaffe E, et al. Microcystic adnexal carcinoma
associated with primary immunodeficiency, recurrent diffuse herpes
simplex virus infection, and cutaneous T-cell lymphoma. \emph{Am
JDermatopathol} 2000;22:524--9.

5. Massey PR, Soldano AC, Fox MC. Adnexal Carcinoma: Microcystic Adnexal
Carcinoma and Sebaceous Carcinoma. A Practical Guide to Skin Cancer:
Springer; 2018. p. 201-30.

6. Chaudhari SP, Mortazie MB, Blattner CM, Garelik J, Wolff M, Daulat J,
et al. Treatments for microcystic adnexal carcinoma--A review. Journal
of Dermatological Treatment. 2016;27(3):278-84.

7. Rongioletti F, Grosshans E, Rebora A. Microcystic adnexal
carcinoma.\emph{Br J Dermatol} 1986;115:101--4.

8. Sebastien TS, Nelson BR, Lowe L. Microcystic adnexal
carcinoma.\emph{J Am Acad Dermatol} 1993;29:840--5.

9. Glatt HJ, Proia AD, Tsoy EA. Malignant syringoma of the
eyelid.\emph{Ophthalmology} 1984;91:987--90.

10. Lipper S, Peiper SC. Sweat gland carcinoma with syringomatous
features: a light microscopic and ultrastructural study.
\emph{Cancer}1979;44:157--63.

11. Ong T, Liew S, Mulholland B, Davis P, Calonje E. Microcystic adnexal
carcinoma of the eyebrow. Ophthalmic Plastic \& Reconstructive Surgery.
2004;20(2):122-5.

12. LeBoit PE, Sexton M. Microcystic adnexal carcinoma of the skin: a
reappraisal of the differentiation and differential diagnosis of an
under recognized neoplasm. \emph{J Am Acad Dermatol} 1993;29:609--18.

13. Sabhiki AK, Clementina RR, Kumar RV, Hazarika D. Microcystic adnexal
carcinoma. \emph{Int J Dermatol} 1997;36:123--44.

14. Yugueros P, Kane WJ, Goellner JR. Sweat gland carcinoma: a
clinicopathologic analysis of an expanded series in a single
institution. Plast Reconstr Surg 1998;102:705--10.

15. Bier-Laning CM, Hom DR, Gapany M, et al. Microcystic adnexal
carcinoma: management options based on long-term
follow-up.\emph{Laryngoscope} 1995;105:1197--201.

16. Hesse R, Scharfenberg J, Ratz J. Eyelid microcystic adnexal
carcinoma. \emph{Arch Ophthalmol} 1995;113:494--6.

17. Burns MK, Chen SP, Goldberg L. Microcystic adnexal carcinoma. Ten
cases treated by Mohs micrographic surgery. J Dermatol Surg Oncol
1994;20:429--34.

18. Billingsley EM, Fedok F, Maloney ME. Microcystic adnexal carcinoma:
case report and review of the literature. Arch Otolaryngol Head Neck
Surg 1996;122:179--82.

19. Hamm JC, Argenta LC, Swanson NA. Microcystic adnexal carcinoma: an
unpredictable aggressive neoplasm. Ann Plast Surg 1987; 19:173--80.

20. Lober CW, Larbig G. Microcystic adnexal carcinoma (sclerosing sweat
duct carcinoma). South Med J 1994;87:259--62.

21. Borenstein A, Seidman DS, Trau H, Tsur H. Case report: microcystic
adnexal carcinoma following radiotherapy in childhood. Am J Med Sci
1991;301:259--61.

22. Snow S, Madjar Jr DD, Hardy S, Bentz M, Lucarelli MJ, Bechard R, et
al. Microcystic adnexal carcinoma: report of 13 cases and review of the
literature. Dermatologic surgery. 2001;27(4):401-8.

23. Carroll P, Goldstein G, Brown CW. Metastatic microcystic adnexal
carcinoma in an immunocompromised patient. Dermatol Surg 2000;26:531--4.

24. Brookes JL, Bentley C, Verma S, Olver JM. Microcystic adnexal
carcinoma masquerading as a chalazion. Br J Ophthalmol 1998;82:196--9.

25. Clement CI, Genge J, O'Donnell BA, Lochhead AG. Orbital and
periorbital microcystic adnexal carcinoma. Ophthal Plast Reconstr Surg.
2005;21:97--102.

26. Barlow RJ, Ramnarain N, Smith N, et al. Excision of selected skin
tumours using Mohs' micrographic surgery with horizontal
paraffinembedded sections. Br J Dermatol. 1996;135:911--17.

27. Leibovitch I, Huilgol SC, Selva D, et al. Microcystic adnexal
carcinoma: treatment with Mohs micrographic surgery. J Am Acad Dermatol.
2005;52:295--300.

28. Lang J, Suzuki S, Honda K, et al. Sweat duct carcinoma of lip with
multiple cervical lymph nodes metastasis. Auris Nasus Larynx.
2006;33:337--41.

29. Abbate M, Zeitouni NC, Seyler M, et al. Clinical course, risk
factors, and treatment of microcystic adnexal carcinoma: a short series
report. Dermatol Surg. 2003;29:1035--8.

30. Chiller K, Passaro D, Scheuller M, et al. Microcystic adnexal
carcinoma: forty-eight cases, their treatment, and their outcome. Arch
Dermatol. 2000;136:1355--9.

31. Stein JM, Ormsby A, Esclamado R, Bailin P. The effect of radiation
therapy on microcystic adnexal carcinoma: a case report. Head Neck.
2003;25:251--4.

32. Yuh WT, Engelken JD, Whitaker DC, Dolan KD. Bone marrow invasion of
microcystic adnexal carcinoma. Ann Otol Rhinol Laryngol.
1991;100:601--3.

33. Beer KT, Buhler SS, Mullis P, et al. A microcystic adnexal carcinoma
in the auditory canal 15 years after radiotherapy of a 12-year-old boy
with nasopharynx carcinoma. Strahlenther Onkol. 2005;181:405--10.

34. Fleischmann HE, Roth RJ, Wood C, Nickoloff BJ. Microcystic adnexal
carcinoma treated by microscopically controlled excision. J Dermatol
Surg Oncol. 1984;10:873--5.

35. Nickoloff BJ, Fleischmann HE, Carmel J, et al. Microcystic adnexal
carcinoma. Immunohistologic observations suggesting dual (pilar and
eccrine) differentiation. Arch Dermatol. 1986;122:290--4.

36. Rotter N, Wagner H, Fuchshuber S, Issing WJ. Cervical metastases of
microcystic adnexal carcinoma in an otherwise healthy woman. Eur Arch
Otorhinolaryngol. 2003;260:254--7.

37. Salerno S, Terrill P. Will MAC be back? ANZ J Surg. 2003;73:830--2.

38. Culhaci N, Copcu HE, Dikicioglu E. Microcystic adnexal carcinoma:
report of a case. J Oral Maxillofac Surg. 2003;61:723--5.

39. Robinson ML, Knibbe MA, Roberson JB. Microcystic adnexal carcinoma:
report of a case. J Oral Maxillofac Surg. 1995;53:846--9.

40. Ongenae KC, Verhaegh ME, Vermeulen AH, Naeyaert JM. Microcystic
adnexal carcinoma: an uncommon tumor with debatable origin. Dermatol
Surg. 2001;27:979--84.

41. Antley CA, Carney M, Smoller BR. Microcystic adnexal carcinoma
arising in the setting of previous radiation therapy. J Cutan
Pathol.1999;26:48--50.

42. Gardner ES, Goldberg LH. Neglected microcystic adnexal carcinoma:
the second reported case in a black patient. Dermatol Surg.
2001;27:678--80.

43. Ohtsuka H, Nagamatsu S. Microcystic adnexal carcinoma: review of 51
Japanese patients. Dermatology. 2002;204:190--3.

44. Schwarze HP, Loche F, Lamant L, et al. Microcystic adnexal carcinoma
induced by multiple radiation therapy. Int J Dermatol. 2000;39:369--72.

45. Mayer MH, Winton GB, Smith AC, et al. Microcystic adnexal carcinoma
(sclerosing sweat duct carcinoma). \emph{Plast Reconstr
Surg}1989;84:970--5.

46. Orentreich N. Autografts in alopecias and other selected
dermatological conditions. Ann N Y Acad Sci 1959;83:463Y479

47. Sharma R, Ranjan A. Follicular Unit Extraction (FUE) Hair
Transplant: Curves Ahead. Journal of Maxillofacial and Oral Surgery.
2019:1-9.

48. Akdag O, Evin N, Karamese M, Tosun Z. Camouflaging cleft lip scar
using follicular unit extraction hair transplantation combined with
autologous fat grafting. Plastic and Reconstructive Surgery.
2018;141(1):148-51.

49. Jung S, Oh SJ, Koh SH. Hair follicle transplantation on scar tissue.
Journal of Craniofacial Surgery. 2013;24(4):1239-41.

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