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Vulvar dermal deep nodule in 31 years old woman.

Giorgio Gherardi

Servizio di Anatomia e Istologia Patologica
Ospedale Fatebenefratelli e Oftalmico

Comentado en:
 Historia Clínica
Excisional biopsy of a vulvar deep dermal nodule in the right labium maius of a 31-years'old woman. The lesion was not painful and was detected by the patient three months earlier. She was seen by a Gynecologist who excised the nodule and sent it for histopathological evaluation. Gynaecological visit and exam were otherwise unremarkable. The clinical history is not contributory.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
Imagen de Vulvar dermal deep nodule in 31 years old woman.


El 5/12/2004 21:39, Daniel Scharifker dijo:

Me inclino por una Carcinoma Adenoideo-quistico


El 5/12/2004 22:23, Reynaldo Falcón-Escobedo dijo:


Elementos epiteliales, mioepiteliales, material tipo membrana basal, estroma conectivo denso con áreas basófilas.


El 5/12/2004 22:34, GUSTAVO SALES BARBOSA dijo:



El 5/12/2004 22:37, Iván Gallegos Méndez. Chile. dijo:

Me parece un siringoma condroide (adenoma pleomorfo) , por su contorno bien delimitado y la citología.


El 5/12/2004 23:18, Reynaldo Falcón-Escobedo dijo:



El 6/12/2004 6:27, metin akbulut dijo:

I am in favour of a chondroid syringoma because of the well-defined nodular architecture and absence of desmoplastic stromal reaction , I do not think it is a malignant tumor


El 6/12/2004 9:23, marco alberghini dijo:

I think that we are dealing with a benign adnexal tumor , and the architecture of the nodule fit well with the diagnosis of chondroid syringoma


El 6/12/2004 10:13, Saba Hoda dijo:

The tumor is rather well defined but unencapsulated. Tumoral cells are basaloid and uniform and are arranged as nests,tubules and microcysts.A cribriform pattern is also seen and hyalinized meterial is evident surrounding some lobules.My diagnosis is cylindroma.


El 6/12/2004 11:45, Flavio O Lima dijo:

Caros colegas

Penso em uma lesão com diferenciação ecrina, que seria melhor classificada por um especialista(não o sou), mas sugiro um Carcinoma Ecrino Adenoide-Cistico.

Belo caso, com belas imagens

Flavio Lima

Avare SP Brasil


El 6/12/2004 16:38, Bayardo Flores dijo:

Si bien a primera vista el tumor pareciera corresponder a un siringoma condroide, el patrón microquístico, la ausencia de mioepitelio y de diferenciación condroide del estroma, así como la localización profunda, me inducen a proponer un Carcinoma Microquístico Anexial (Microcystic Adnexal Carcinoma). Poco frecuente y aún menos frecuente en la vulva.

Saludos pre-navideños.


Locarno, Suiza


El 6/12/2004 17:01, Francisco Paz Gomez México DF dijo:



El 6/12/2004 23:27, Francisco Mota dijo:

Carcinoma Adenoideo quìstico


El 7/12/2004 1:02, Myriam Catalina dijo:

Por la buena delimitación de la lesión y las características citológicas creo que podría tratarse de un siringoma condroide


El 7/12/2004 6:37, Clelia Miracco dijo:

Thank you for the new case. The tumor is unencapsulated, although well demarcated. It seems to be characterized by "adenoid", trabecular, cord, and solid arrangements; in some areas structures similar to "eccrine ducta" are shown. the tumor cells are polygonal with eosinophilic cytoplasm, sometimes with prominent nucleoli; clear cells are also evident. The stroma is hyaline, sometimes myxoid. There is strong eosinophilic material inside few "luminal" structures.

I would consider the following differential diagnoses:

!- myoepithelioma, such as the one described by Fukunaga, Apmis Volume 111 Issue 3 Page 416 - March 2003

!!- adenomyoepithelioma, as the ones described in the breast, etc (in some areas of fig.5 there are some structures similar to ducta, in which dual layered gland-like structures and an epithelial component seem to be shown; furthermore, there are "apocrine" features in some "lumina". (To be honest, I favour this second hypothesis!)

The appearance of the lesion seems benign. I think it would be helpful immunohistochemical investigation for:

vimentin, alpha-smooth actin, GFAP, S-100, Cytokeratins (14 and other fractions), MIB1.

Clelia Miracco

Anatomia Patologica, Siena- Italy


El 7/12/2004 8:24, vincenzo polizzi dijo:

Un caro saluto a tutti. Questo è un caso interessante perchè mi sembra maligno, tipo carcinoma delle ghiandole del Bartolino, ma è ben delimitato...potrebbe essere una METASTASI DI CARCINOMA MICROPAPILLARE DELLA MAMMELLA.

Vincenzo Polizzi


El 7/12/2004 16:52, Armando Rossi dijo:

In my opinion the microphotograph appears to show diagnostic features of apocrine mixed tumor.


El 7/12/2004 18:29, Dan Pankowsky dijo:

I favor that this is an ectopic breast which shows ductal carcinoma in situ vs. low grade invasive ductal carcinoma of the "breast".


El 7/12/2004 21:09, Túlio Souza dijo:


In my point of view the pictures are very similar to a breast biopsy.

I thing it’s a breast carcinoma arising in ectopic mammary tissue the main differential diagnostic is carcinomas of sweat glands.



El 7/12/2004 22:29, Veronica Resende Lima dijo:

me parece um carcinoma adenóide-cístico de glândulas écrinas


El 8/12/2004 11:46, Celso Rubens Vieira e Silva dijo:

I agree with Dr Tulio, the pictures looks-like well differentiated mammary carcinoma. In the last immage we see also a typical "intra cytoplasmatic lumen" inclusion.The literature includes in the list of epithelial vulvar lesions, a rare tumor with " breast-like " features, originating probably in anogenital adnexal glands.

1: Ann Pathol. 1999 Apr;19(2):124-7.

[Breast-like carcinoma of the vulva]

Erb-Gremillet S, Gunther M, Amiaux F, Parache RM.

Service d'Anatomie et de Cytologie Pathologiques, Centre Alexis-Vautrin (CRLCC),


Breast-like carcinomas of the vulva is a rare finding. Only 11 cases are

reported in the literature. This article reports a painful tumor of the left

vulvar labia in a 62 year-old woman. Excisional biopsy showed an infiltrating

adenocarcinoma, histologically similar to a breast tumor with positive hormonal

receptors. The origin of this tumor, ectopic mammary tissue or specific adnexal

genito-anal gland, is discussed. From data of the literature, we offer

guidelines for diagnosis, treatment and origin of this rare tumor.

PMID: 10349477 [PubMed - indexed for MEDLINE]

2: Eur J Gynaecol Oncol. 2002;23(4):350-2.

Breast-like cancer of the vulva: primary or metastatic?

A case report and review of the literature.

Miliaras D.

Pathology Department, General Clinic, Thessaloniki, Greece.

A 45-year-old white female presented a polypoid nodule in the vulva, one year

after she was operated on for breast cancer. Histologic examination showed a

poorly differentiated carcinoma that closely resembled the primary breast tumor.

Eight similar cases have been previously described in the literature. This very

rare event should be differentiated from primary adenocarcinoma of the

mammary-like glands of the vulva. The recognition of such a lesion as primary or

metastatic is very important, since it greatly influences management and

prognosis.PMID: 12214743 [PubMed - indexed for MEDLINE]

3: Wien Klin Wochenschr. 2000 Oct 13;112(19):855-8.

Primary breast cancer of the vulva: a case report and review of the literature.

Gorisek B, Zegura B, Kavalar R, But I, Krajnc I.

Clinical Department of Gynecology and Perinatology, Maribor Teaching Hospital,

Slovenia. borut.gorisek@sb-mb.si

Since 1872, 40 cases of ectopic mammary gland tissue in the vulva have been

reported in the literature. Out of these, 12 had a primary cancer in the ectopic

breast tissue. Seven metastases of an orthotopic breast cancer have been found

in this location. We are presenting the 20th case of cancerous breast tissue in

the vulva whom we classified as the 13th case of primary cancer based on

clinical and histopathological criteria of primary and metastatic malignant

disease. Because of the advanced age of the patient, wide local excision

followed by adjuvant hormonal therapy was opted for. Nineteen months after

surgery, there is no evidence of recurrent disease. Due to the rarity of this

entity, its management presents therapeutic dilemmas, and variable treatment

strategies are being found in the literature. In our opinion, the same basic

principles used for treatment of cancers of the orthotopic breast should be

applied in ectopic breast carcinoma.PMID: 11098538 [PubMed - indexed for MEDLINE]

4: Eur J Gynaecol Oncol. 2002;23(1):21-4.

Primary breast carcinoma of the vulva: case report and review of literature.

Piura B, Gemer O, Rabinovich A, Yanai-Inbar I.

Department of Obstetrics and Gynecology, Soroka Medical Center and Faculty of

Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel.

The occurrence of ectopic breast tissue within the vulva is uncommon and the

development of breast carcinoma within vulvar ectopic breast tissue is very

rare. To date, only 12 cases of primary vulvar breast carcinoma have previously

been reported in the English literature. This paper presents the 13th reported

case of primary breast carcinoma of the vulva. The patient presented with a

vulvar ulcerated lump and the diagnosis was based on a morphologic pattern

consistent with breast carcinoma and the presence of estrogen and progesterone

receptors. Primary surgery consisted of radical vulvectomy and bilateral groin

dissection. The groin lymph nodes were involved bilaterally. Adjuvant therapy

consisted of systemic chemotherapy (4 cycles of adriamycin and cyclophosphamide

followed by 4 cycles of paclitaxel) and pelvic radiotherapy. Oral tamoxifen 20

mg/day was started for the next five years. It is concluded that the management

of primary breast carcinoma of the vulva should be modeled after that for

primary carcinoma of the orthotopic breast with primary surgery followed by

systemic chemotherapy and pelvic radiotherapy. Chemotherapy should be similar to

that employed for breast carcinoma. Tamoxifen should be prescribed for patients

whose tumors contain estrogen receptors.PMID: 11876386 [PubMed - indexed for MEDLINE]

5: Rev Med Chil. 2001 Jun;129(6):663-5.

[Mammary carcinoma ine ectopic breast tissue. A case report]

Pardo M, Silva F, Jimenez P, Karmelic M.

Unidad de Mastologia, Departamento de Obstetricia y Ginecologia, Hospital

Clinico de la Universidad de Chile, Av. Santos Dumont 999, Santiago-Chile.

Ectopic breast tissue, that includes supernumerary breasts and aberrant breast

tissue, develops along the mammary line. Malignancies rarely develop in this

tissue. We report a 44 years old female subjected to a resection of a 3 cm O

tumor located 2 cm below the right lower mammary sulcus. The pathological study

reported an infiltrating ductal carcinoma. The patient was operated again and a

metastatic carcinoma was detected in three resected axillary lymph nodes.

Adjuvant chemotherapy and radiotherapy was indicated.PMID: 11510208 [PubMed - indexed for MEDLINE]


I think that we need to make the differential diagnosis with “Microcystic adnexal carcinoma of the vulva” by immunohistochemistry study.


Microcystic adnexal carcinoma of the vulva

Gynecol Oncol. 2001 Sep;82(3):571-4.

Buhl A, Landow S, Lee YC, Holcomb K, Heilman E, Abulafia O.

Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, State

University of New York Health Science Center at Brooklyn, 450 Clarkson Avenue,

Brooklyn, New York, 11203, USA.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a subset of sweat gland

carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr,

and D. J. Santa Cruz (Cancer 1982;50:566-72). We report the first case of MAC

occurring on the vulva and review the literature pertaining to this rare tumor.

CASE: A 43-year-old multiparous black woman presented initially to Kings County

Hospital Medical Center with a chief complaint of a vulvar lesion arising on the

left labia majora which she had noted for 4 years prior to presentation. Aside

from increasing paresthesia in the area, she denied any constitutional symptoms.

Her past medical history was significant only for hyperthyroidism and mild

hypertension and surgical history was noncontributory. Gynecologic history was

unremarkable, with sporadic care over the last 20 years. Physical examination

revealed a 1.5 x 2.0-cm raised, well-circumscribed, firm mobile lesion on the

left labia majora. It was noted to be yellow in color with the surrounding

tissue being unremarkable in character. The remainder of her gynecologic

examination and lymph node survey was unremarkable. Preoperative chest X ray was

negative as was the CAT scan of the abdomen and pelvis. All laboratory values

were within normal limits. A Pap smear done preoperatively was significant for

atypical squamous and glandular cells of undetermined significance. Subsequent

colposcopic examination of the cervix was remarkable for cervicitis and was

adequate, with the entire transformation zone visualized. Both endocervical

curettage and endometrial biopsy were normal. Initially, an excisional biopsy

was performed with final pathology demonstrating microcystic adnexal carcinoma

with positive surgical margins. She subsequently underwent a left radical

hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time

of surgery, the left labia majora was noted to be well healed, with a residual

surgical scar easily discernible. No areas of discoloration were noted and

digital palpation of the area was unremarkable. Microscopic residual tumor was

noted; however, all surgical margins and lymph nodes were negative for tumor.

Her postoperative course was unremarkable. The patient has continued to do well

since the time of her surgery and is being followed conservatively. CONCLUSION:

Radical vulvectomy should be performed when MAC occurs in the vulva to secure

negative margins of resection. Groin dissection should be reserved for cases in

which the inguinal lymph nodes are clinically suspicious or in cases of tumor

recurrence. PMID: 11520158 [PubMed - indexed for MEDLINE]


I beg to be excused, by the long text…




Hospital Aliança

Salvador Bahia Brasil.



El 8/12/2004 14:45, Luis Muñoz Fernández dijo:

Caso difícil de analizar por tener elementos aparentemente contradictorios: se trata de una mujer joven, con lesión bien circunscrita, se observan una proliferación epitelial con un estroma llamativo, basófilo, un tanto mixoide, sin desmoplasia significativa. Dado que no podemos ver con detalle la morfología celular, tenemos limitaciones para conocer el grado de atipia, aunque parece bajo. La disposición de las células es en nidos y glándulas de luces muy redondas "rígidas"). Si intento conciliar los aspectos morfológicos de "benignidad" con los de "malignidad", creo que una buena posibilidad es un adenocarcinoma de grado bajo, que bien puede haverse originado en tejido mamario primario o metastásico en la vulva. De cualquier modo, no deja de ser una hipótesis por lo pronto.


El 8/12/2004 18:00, Victor Prieto dijo:

Dear Colleagues:

Thank you very much for posting this very interesting case. From the long list of diagnoses posted by the participants, it is evident that this is not an easyly diagnosable case. In my opinion, even though the lesion appears "nodular" and has been interpreted as a benign adnexal tumor by some participants, I believe it has an infiltrative pattern at the periphery. That fact, along with the cytology displayed in the provided microphotograps, supports a diagnosis of adenocarcinoma. Being from this area, I think that this is consistent with an adnexal apocrine carcinoma, probably arising from the mammary-like glands located in this anatomic location. In order to support that diagnosis, the contributor may want to investigate the presence of myoepithelial cells in the lesion.


Victor G. Prieto


El 8/12/2004 22:14, Alberto Giménez dijo:

A pesar de las interesantes opiniones aqui expresadas, creo, como dice el viejo aforismo, que si se ve un pájaro a lo lejos, sobre una chimenea, y en Madrid, es mucho más probable que se trate de un gorrión que de un papagayo...Por tanto, me sumo a los que creen que este tumor es, muy probablemente,un siringoma condroide (tumor mixto benigno, adenoma pleomorfo). Saludos a todos.

In spite of the interesting opinions here expressed, I believe, as says the old aphorism, which if one sees a bird in the distance, on a chimney, in Madrid, is much more probable that it is a sparrow that a parrot... Therefore, I add to those who believe that this tumor is, very probably,a chondroid syringoma (benign mixed tumor, pleomorphic adenoma). Regards.


El 9/12/2004 3:30, Hernan Molina Kirsch dijo:

Es una lesion bien delimitida y sus celulas epiteliales son citologicamente blandas como para favorecer malignidad por lo que me inclino a favor de Tumor Mixto o Siringoma.

Hernan Molina Kirsch



El 9/12/2004 9:33, Miguez Sanz-Anquela dijo:

La lesión presenta un patrón típicamente cribiforme que sugiere un carcinoma adenoide quístico, si bien

las luces del carcinoma adenoide quístico son por lo general más grandes, redondeadas y regulares, de lo

que puede observarse en este bonito caso. La celularidad es monomorfa y con citoplasma eosinófilo de

aspecto apocrino, que ha sido sugerido también por algunos compañeros. Este patrón puede observarse en

lesiones mamarias por lo que algunos colegas piensan se ha originado en glándula mamaria ectópica.

En mi opinión, las imágenes presentadas de este caso cumplen los parámetros morfológicos y clínicos del

carcinoma cribiforme cutáneo. Incluso el aspecto mixoide del estroma que rodea inmediatamente al

componente epitelial, y que sustenta el diagnóstico de tumor mixto, se ha descrito también en algunos

carcinomas cribiformes cutáneos.

El carcinoma cribiforme cutáneo es una rara variante histológica de probable diferenciación apocrina

descrita en la monografía sobre Neoplasias con Diferenciación Apocrina, de Requena, Kiryu y Ackerman

(Lippincott-Raven, 1998) y sin otras referencias hasta la actualidad salvo el capítulo que dedica el

propio Luis Requena en su reciente libro sobre neoplasias anexiales cutáneas:


Un cordial saludo a todos desde Alcalá de Henares.

Sanz-Anquela JM

Servicio de Anatomia Patologica

Hospital Principe de Asturias

Campus Universitario

28805 Alcala de Henares (Madrid)


(transcrito desde la lista de mail dermopat@uninet.edu, pues erroneamente fué escrito alli)


El 9/12/2004 16:50, Maria Laura Fibbi dijo:

I think I am dealing with a malignant tumor, and I think it is an adenoid cystic carcinoma

Maria Laura Fibbi


El 10/12/2004 1:50, Sandro Casavilca dijo:

Nodulo bien delimitado en el que se observan grupos de células epiteliales con un patron predominantemente cribiforme y estroma de aspecto mixoide.

Los cambios estromales y las características arquitecturales de la lesión, así como la ausencia de compromiso perineural me inclinan a favorecer el diagnóstico de siringoma condroide- tumor mixto.


El 10/12/2004 9:57, MAURIZIO SPINELLI dijo:

I thing it’s a Adenomyoepithelioma arising in ectopic breast tissue.

Un caro saluto a tutti e complimenti a Giorgio per il bellissimo e complicato caso.

Maurizio Spinelli-Milano


El 14/12/2004 23:00, Raquel dijo:

Meu primeiro pensamento foi Carcinoma ductal da mama. origem? Adorei o longo comentário do Celso

abraço a todos

Raquel Tatuí SP BR

Hacer un comentario a este caso
 Comentario del Autor
The lesion consisted of a locally infiltrative nodular tumor composed of solid branching cords and nests variously mixed with tubules and ductular structures. The cords contained optically clear vacuolar or microcystic spaces and the tubules, or ducts, had an empty lumen and were delimited by cuboidal cells. There was only one type of proliferating cells and they were characterized by scanty cytoplasm and a vesicular nucleus with occasional small nucleoli. Mitoses and apoptotic changes were only occasionally seen. The stroma was basophilic with spindle cells or appeared homogeneous and eosinophilic Figura_4 . No signs of squamous-cell or pilar differentiation were evident. At the periphery of the nodule the cords appeared to grow in a definitely infiltrative pattern. The skin surrounding the excised tumor showed only normal eccrine adnexal structures. The tumor cells were strongly immunoreactive for CK7 and less so for S100 protein. EMA Figura_6 and polyclonal CEA Figura_7 immunostains helped decorate the intracytoplasmic lumina within cords and the luminal borders of cells within ducts. These spaces contained a variable amount of PAS-positive, diastase-resistant and Alcian-Blu pH 2.5 positive mucin. No actin-immunoreactive myoepithelial cells could be distinguished within the epithelial lesional tissue while stromal spindle cells were immunopositive for this marker Figura_8 . There was no accumulation of basal lamina material as demonstrated by a negative reaction for collagen type IV. Immunostaining for GFAP, CK20, Estrogen and Progesterone Receptors, and HER2/neu gene product gave negative results.
The architecture of the tumor, the morphology of proliferating cells, and the immunohistochemical results (in particular the EMA and CEA pattern of reaction) are strongly in favor of the diagnosis of eccrine ductal adenocarcinoma 1 . This is a rare tumor that prevails in the head and neck region of elderly patients 1 , but it can occur at any age and site, including the vulva 1 , 2 . By definition the tumor is to be differentiated from a metastatic adenocarcinoma 3 . In the current case, as suggested in several comments, the tumor could be interpreted as a metastatic deposit of a breast carcinoma but we excluded this possibility based on the clinical data and the lack of ER, PgR or HER2/neu gene product cellular expression. On the same basis we also excluded a primary adenocarcinoma of anogenital mammary-type glands 4 which are expected to show hormonal receptor expression especially in well differentiated tumors 5 . Other primaries (intestinal, endocervical) seemed unlikely. The Bartholin glands seemed unaffected clinically.
In conclusion, the diagnosis of eccrine ductal adenocarcinoma was confirmed by Juan Rosai, M.D., who examined the slides in a collegial review session at the Istituto Nazionale Tumori in Milan. A wider excision of the lesion was recommended in the pathology report but we are unaware of the subsequent evolution of the case.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
Imagen de Vulvar dermal deep nodule in 31 years old woman.
1 Urso C, Bondi R, Paglierani M, Salvadori A, Anichini C, Giannini A Carcinomas of sweat glands. Report of 60 cases. Arch Pathol Lab Med 125; 498-505, 2001.
2 Wick MR, Goellner JR, Wolfe JT, Su WPD Vulvar sweat gland carcinoma. Arch Pathol Lab Med 109: 43-47, 1985.
3 Wick MR, Ockner DM, Mills SE, Ritter JH, Swanson PE Homologous carcinomas of the breasts, skin, and salivary glands. A histologic and immunohistochemical comparison of ductal mammary carcinoma, ductal sweat gland carcinoma and salivary duct carcinoma. Am J Clin Pathol 109: 75-84, 1998
4 van der Putte SC, van Gorp LH Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget’s disease. J Cutan Pathol 21: 157-163, 1994
5 Miliaras D. Breast-like cancer of the vulva primary or metastatic? A case report and review of the literature. Eur J Gynaecol Oncol 23: 350-352, 2002
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