Cup 13417

Received: 18 August 2018 Revised: 6 December 2018 Accepted: 11 December 2018
DOI: 10.1111/cup.13417
COVER QUIZLET
Guilherme Muzy, Nilton Gioia Di Chiacchio
Figures 1 and 2 are depicted on the journal cover.
FIGURE 4
FIGURE 3 FIGURE 5
FIGURE 3
Your diagnosis?
Discussion follows on page 302
© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
J Cutan Pathol. 2019;46:301–304. wileyonlinelibrary.com/journal/cup 301
302 MUZY AND DI CHIACCHIO
Periungual angioleiomyoma: A rare tumor

Guilherme Muzy MD1 | Nilton Gioia Di Chiacchio MD2
1
Department of Dermatology, The ABC Foundation School of Medicine, São Paulo, Brazil
2
Department of Dermatology, Hospital do Servidor Público Municipal, São Paulo, Brazil
Correspondence
Guilherme Muzy, MD, Department of Dermatology, The ABC Foundation School of Medicine, São Paulo, Brazil
Email: guimuzy@gmail.com
K E Y W O R D S : cutaneous neoplasm, dermatopathology, skin cancer, soft tissue tumors, atypical features
Angioleiomyomas are one of the 3 variants of leiomyoma and are a case of a 2 cm painful nodule on the great toe. It was diagnosed
benign neoplasms that arise from smooth muscle of veins. The other histologically as an angioleiomyoma with significant myxoid degen-
two are piloleiomyoma, which arises from arrector pili muscle, and eration. In 2000, Baran8 reported a case of an angioleiomyoma of
genital leiomyoma, which arises from smooth muscle of the vulva, the right index finger, initially diagnosed as a glomus tumor due to
scrotum and nipple.1,2 sensitivity to cold and associated tenderness. Histopathological
Typically, it appears on the extremities,3 but cases with facial, examination was compatible with angioleiomyoma, and immunohis-
4,5
truncal or even oral involvement have been reported. Previous tochemical study demonstrated that the spindle cells expressed
reports of subungual and periungual angioleiomyomas are quite rare. immunoreactivity for vimentin, alpha smooth muscle actin and des-
6
In 1889, Lebouc was the first to describe a case of subungual leio- min. In 2017, Wakada9 reported a case of a 40-year-old woman with
7
myoma of the great toe, followed by Sawada in 1988, who reported a 5-month history of nail changes and tenderness of the left index
FIGURE 1 A: Lateral view of the tumor. B: Immediately after resection

MUZY AND DI CHIACCHIO 303
FIGURE 3 Scanning view of the nodule, with fascicles of smooth

muscle admixed with well-developed blood vessels, with
unremarkable epidermis (H&E ×40)
FIGURE 4 1-year follow-up with mild-to-moderate
onychodystrophy
FIGURE 5 X-ray scan, unremarkable
angioleiomyomas are painful, and conversely, pain is a common symp-

tom of other cutaneous neoplasms such as glomus tumors, spiradeno-
mas, angiolipomas or neuromas. Therefore, the diagnosis of
angioleiomyoma usually rests on histopathological examination, since
even imaging modalities are non-specific.9 Morimoto10 classified
angioleiomyomas into three histological types: solid, venous, and cav-
ernous. The solid-type tumors consisted of intersecting smooth mus-
cle bundles surrounding numerous small vascular channels. The
cavernous-type tumors consisted of dilated vascular channels, with
smaller amounts of smooth muscle. The venous-type tumors pre-
sented thick-walled venous channels and less-compacted smooth
muscle bundles.11
FIGURE 2 A: Well demarcated nodule with smooth muscle fibers
From a histopathological point of view, the main differential
(H&E ×100). B: Note the differently sized vessels (H&E ×400)
diagnoses are glomus tumor and angioleiomyolipoma. The former
can be easily distinguished from angioleiomyoma by the presence of
finger. Histological examination revealed a fibrillar encapsulated glomus cells, while in the latter, fat cells are easily identifiable in rou-
tumor composed of fascicles of spindle cells with a complex arrange- tine hematoxylin and eosin (H&E) examination. Table 1 summarizes
ment. Immunohistochemistry showed that the spindle cells were the histopathological findings of the most common differential
positive for alpha-smooth muscle actin and desmin, findings consis- diagnoses.
tent with an angioleiomyoma.
TABLE 1 Differential diagnosis and histopathology
A 70-year-old male patient had a 3-year history of a slowly grow-
Tumor Histopathological findings
ing nodule on the 5th right toe (Figure 1A). He denied experiencing Glomus tumor Perivascular round cells with bland nuclei,
trauma and bleeding but experienced discomfort and mild pain when vessels of different caliber, rare mitoses and
no atypia
wearing shoes. Physical examination revealed a 2 cm violaceous exo-
Angioleiomyoma Encapsulated, rounded contour, and blood
phytic nodule on the proximal nail fold of the 5th right toe, and no vessels interlacing smooth muscle fibers.
changes on the corresponding nail were observed. Leiomyosarcoma Infiltrative growth pattern, atypical smooth
A surgical approach was performed. A distal block with 1% lido- muscle cells and numerous mitoses
caine was used, and along with a tourniquet, an oblique incision Dermatomyofibroma Fascicles of spindled cells parallel to the
epidermis, cigar-shaped nuclei and
enabled the lesion to be easily removed down to the level of the nail
eosinophilic cytoplasm, no atypia or mitoses.
matrix (Figure 1B). The wound healed by second intention.
Microscopic examination demonstrated a sharply demarcated
nodule in the dermis, surrounded by fibrous tissue. The nodule was
In conclusion, periungual angioleiomyoma is a rare tumor with no
composed of different types of blood vessels and smooth muscle fas-
distinct clinical features, but it should be included in the differential
cicles. These blood vessels had lumina of varying size, and some were
diagnosis of periungual and subungual neoplasms. Surgical excision is
rounded, while others were slit-like. The cells in the smooth muscle
usually a definite treatment and a precise diagnosis is possible with
fascicles contained blunt-tipped, straight nuclei and abundant eosino-
routine H&E staining.
philic cytoplasm. Neither fat tissue nor glomus cells were present in
the tumor. There was also no evidence of necrosis, pleomorphism,
mitoses or nuclear atypia. Thus, the diagnosis of angioleiomyoma was ORCID
made (Figures 2 and 3). After a 1-year follow-up, the patient devel- Guilherme Muzy https://orcid.org/0000-0002-7302-4113
oped mild-to-moderate onychodystrophy but was satisfied with the Nilton Gioia Di Chiacchio https://orcid.org/0000-0001-5944-7737
overall result (Figure 4). No recurrence was observed. An x-ray scan
demonstrated swelling of soft tissue, with no changes on the underly-
RE FE RE NC ES
ing bone or joint (Figure 5).
1. Fisher WC, Helwig EB. Leiomyomas of the skin. Arch Dermatol. 1963;
Periungual angioleiomyoma is a rare neoplasm that has no distinc-
88:510-520.
tive clinical features. Pain and tenderness are the only clinical findings 2. Montgomery H, Winkelmann RK. Smooth-muscle tumors of the skin.
which could suggest the nature of the lesion.8 However, not all Arch Dermatol. 1959;79:32-41.
304 MUZY AND DI CHIACCHIO
3. Duhig JT, Ayer JP. Vascular leiomyoma. Arch Pathol. 1959;68:424-430. 9. Watabe D, Sakurai E, Mori S, Akasaka T. Subungual angioleiomyoma.
4. Hachisuga T, Hashimoto H, Enjoji M. Angioleiomyoma: A clinical Indian J Dermatol Venereol Leprol. 2017;83:74-5
reappraisal of 562 cases. Cancer. 1984;54:126-130. 10. Moritmoto N. Angiomyoma (vascular leiomyoma): A clinicopathologic
5. Reichart P, Reznik-Schuller H. The ultrastructure of an oral angio- study. Medical Journal of Kagoshima University. 1973;24:663-683.
myoma. J Oral PathoI. 1977;6:25-34. 11. Lawson GM, Salter DM, Hooper G. Angioleiomyomas of the hand: a
6. Lebouc L. Étude clinique et anatomique sur quelques cas de tumeurs report of 14 cases. J Hand Surg (Br). 1995;20:479-483.
sous-unguéales. Thèse pour le Doctorat en Médecine. Paris: Steinsheil G.
1889: 1–34.
7. Sawada Y. Angioleiomyoma masquerading as a painful ganglion of the How to cite this article: Muzy G, Di Chiacchio NG. Periun-
great toe. Eur J Plast Surg. 1988;11:175–177. gual angioleiomyoma: A rare tumor. J Cutan Pathol. 2019;46:
8. Baran R, Requena L, Drapé JL. Subungual angioleiomyoma masquerad-
301–304. https://doi.org/10.1111/cup.13417
ing as a glomus tumour. Br J Dermatol. 2000;142:1239-1241.

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Received: 18 August 2018 Revised: 6 December 2018 Accepted: 11 December 2018

Guilherme Muzy, Nilton Gioia Di Chiacchio

Figures 1 and 2 are depicted on the journal cover.

Discussion follows on page 302

Periungual angioleiomyoma: A rare tumor

FIGURE 1 A: Lateral view of the tumor. B: Immediately after resection

FIGURE 3 Scanning view of the nodule, with fascicles of smooth

angioleiomyomas are painful, and conversely, pain is a common symp-

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