Abstracts
Granular cell tumor is a rare benign neoplasm of neural origin. We report the case of a female patient, 27 years old presenting a brown-red nodule in the right arm, which pathological examination showed to be formed by polygonal cells with eosinophilic granular cytoplasm and immunohistochemistry positive for S100 protein and CD68. Granular cell tumor is usually solitary and in half the cases located in the head and neck areas, 30% of these in the tongue. It is most frequent between the third and fifth decades of life in women and people of African-American ethnicity. Its origination is controversial, including the possible origins in muscle, fibroblasts, neural crest, neural sheath or histiocytes. The positivity for S-100 and CD68 suggest the neural origin.
Granular cell tumor; S100 Proteins; Schwann cells
O tumor de células granulares é uma neoplasia benigna rara, de origem neural. Relatamos caso de paciente feminina, 27 anos, com nódulo de superfície acastanhada no braço direito, cujo exame anatomopatológico evidenciou densa proliferação de células, com amplo citoplasma contendo grânulos eosinofílicos, e imuno-histoquímica positiva para proteínas S100 e CD68. O tumor de células granulares é geralmente solitário e, em metade dos casos, localiza-se em cabeça e pescoço, dos quais 23% na língua. É mais frequente entre a terceira e a quinta décadas de vida, em mulheres e pessoas de etnia negra. A positividade para S-100 e CD68 favorece origem neural.
Células de Schwann; Proteínas S100; Tumor de células granulares
CASE REPORT
Twenty-seven year old female patient, phototype V, presented a lesion in the right arm for one and a half years, without associated symptoms. Dermatological examination revealed a smooth-surfaced, brownish, painless nodule, movable within deep layers, measuring three centimeters and located in the right arm (Figure 1). Pathological examination of the surgical specimen showed dense proliferation of large cells with granular-appearing eosinophilic cytoplasm, distinct membranes, central or eccentric nuclei and slightly conspicuous nucleoli. The neoplastic cells formed syncytia in some areas and were inserted within the interstices of a desmoplastic stroma, with scarce focal lymphocytic infiltration. The results of immunohistochemical examination were positive for S100 protein, CD63 and CD68 (Figure 2).
Smooth-surfaced hyperchromic nodule, movable in the deep layers, located on the right cubital fossa
Hematoxilineeosin stained fragment, histopathological exam showing dense proliferation of cells with a wide eosinophilic cytoplasm, central nuclei, and slightly conspicuous nucleoli. Immunohistoc hemistry positive for protein S100 e CD68
DISCUSSION
Granular cell tumor (GCT), also known as Abrikossoff tumor, is a relatively rare benign
condition that originates in Schwann cells and presents clinically as a slow-growing,
painless, solitary nodule, that in half the cases is located in the head and neck areas,
including 23% of these in the tongue.11. Ordóñez NG, Mackay B. Granular cell tumor: a review of the pathology
and histogenesis. Ultrastruct Pathol. 1999;23:207-22.,22. Levavi H, Sabah G, Kaplan B, Tytiun Y, Braslavsky D, Gutman H.
Granular cell tumor of the vulva: six new cases. Arch Gynecol Obstet.
2006;273:246-9. GCT can affect patients
of all ages, races and genders, but is more frequent between the third and fifth decades
of life in women and people of African-American ethnicity.22. Levavi H, Sabah G, Kaplan B, Tytiun Y, Braslavsky D, Gutman H.
Granular cell tumor of the vulva: six new cases. Arch Gynecol Obstet.
2006;273:246-9. This tumor was first described in 1926 by Abrikossoff; however,
its pathogenesis has been the object of research and debate for many years. In 1935,
Feyrter suggested a neural differentiation. In the 1960s, clinical and pathologic
origins from Schwann cells were reported.11. Ordóñez NG, Mackay B. Granular cell tumor: a review of the pathology
and histogenesis. Ultrastruct Pathol. 1999;23:207-22.,22. Levavi H, Sabah G, Kaplan B, Tytiun Y, Braslavsky D, Gutman H.
Granular cell tumor of the vulva: six new cases. Arch Gynecol Obstet.
2006;273:246-9. Clinically, it appears
as an asymptomatic dermal or subcutaneous nodule, skincoloured or brown-red, ranging
from 0.5 to 3 centimeters in diameter, with slow growth and generally benign
behavior.33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6.
4. Sehgal VN, Verma P, Sharma S. An asymptomatic, progressive,
ulcerative nodule over the anterolateral surface of the left upper arm. Clin Exp
Dermatol. 2012;37:695-7.-55. Brandão M, Domenech J, Noya M, Sampaio C, Almeida MVC, Guimarães MS,
et al.Tumor de células granulares no pé (tumor de Abrikossoff): localização
infreqüente de tumor relativamente raro (Foot granular cell tumor (Abrikossoff's
tumor): unusual location of a relatively uncommon tumor). An Bras Dermatol.
2001;76:215-222. Some patients may have warty, itchy or painful lesions.33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6. Half of these tumors develop in the head and neck
regions, with the tongue as the most common site.11. Ordóñez NG, Mackay B. Granular cell tumor: a review of the pathology
and histogenesis. Ultrastruct Pathol. 1999;23:207-22.
2. Levavi H, Sabah G, Kaplan B, Tytiun Y, Braslavsky D, Gutman H.
Granular cell tumor of the vulva: six new cases. Arch Gynecol Obstet.
2006;273:246-9.-33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6. However, any organ or
tissue can be affected, such as the digestive and respiratory tracts, pituitary and
parotid glands, skeletal muscles and eyes.33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6.,55. Brandão M, Domenech J, Noya M, Sampaio C, Almeida MVC, Guimarães MS,
et al.Tumor de células granulares no pé (tumor de Abrikossoff): localização
infreqüente de tumor relativamente raro (Foot granular cell tumor (Abrikossoff's
tumor): unusual location of a relatively uncommon tumor). An Bras Dermatol.
2001;76:215-222. Multiple lesions occur
in 5-25% of cases and reports in children are rare.33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6. Histologically, the dermis shows polygonal cells with abundant granular
eosinophilic cytoplasm, central or eccentric vesicular nucleus containing diastase
resistant, and PAS positive granules. Cellular boundaries are usually inaccurate,
resembling a syncytium.44. Sehgal VN, Verma P, Sharma S. An asymptomatic, progressive,
ulcerative nodule over the anterolateral surface of the left upper arm. Clin Exp
Dermatol. 2012;37:695-7. Under electron
microscope, degenerated myelinated axons are seen in the cytoplasm of these tumor
cells.33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6. Positive expression of S-100, CD 68,
CD57, neuron-specific enolase and vimentin confirms the neural origin, although the
classic form of this tumor does not carry any resemblance to other tumors of the
peripheral nerve sheath.33. Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case
report and review of the literature. Cutis. 2002;69:343-6.,44. Sehgal VN, Verma P, Sharma S. An asymptomatic, progressive,
ulcerative nodule over the anterolateral surface of the left upper arm. Clin Exp
Dermatol. 2012;37:695-7.,66. Rekhi B, Jambhekar NA. Morphologic spectrum, immunohistochemical
analysis, and clinical features of a series of granular cell tumors of soft tissues:
a study from a tertiary referral cancer center. Ann Diagn Pathol.
2010;14:162-7.,77. Torrijos-Aguilar A, Alegre-de Miquel V, Pitarch-Bort G,
Mercader-García P, Fortea-Baixauli JM.. Cutaneous granular cell tumor: a clinical and
pathologic analysis of 34 cases. Actas Dermosifiliogr.
2009;100:126-32. Epidermis may present
pseudoepitheliomatous hyperplasia.77. Torrijos-Aguilar A, Alegre-de Miquel V, Pitarch-Bort G,
Mercader-García P, Fortea-Baixauli JM.. Cutaneous granular cell tumor: a clinical and
pathologic analysis of 34 cases. Actas Dermosifiliogr.
2009;100:126-32. Malignant
cases represent 12% and usually have a poor prognosis. Differential diagnoses include
melanocytic nevus, dermatofibromas and adnexal tumors. Treatment involves complete
excision, because if incompletely removed the local recurrence rate is high.44. Sehgal VN, Verma P, Sharma S. An asymptomatic, progressive,
ulcerative nodule over the anterolateral surface of the left upper arm. Clin Exp
Dermatol. 2012;37:695-7.,55. Brandão M, Domenech J, Noya M, Sampaio C, Almeida MVC, Guimarães MS,
et al.Tumor de células granulares no pé (tumor de Abrikossoff): localização
infreqüente de tumor relativamente raro (Foot granular cell tumor (Abrikossoff's
tumor): unusual location of a relatively uncommon tumor). An Bras Dermatol.
2001;76:215-222.
REFERENCES
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1Ordóñez NG, Mackay B. Granular cell tumor: a review of the pathology and histogenesis. Ultrastruct Pathol. 1999;23:207-22.
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2Levavi H, Sabah G, Kaplan B, Tytiun Y, Braslavsky D, Gutman H. Granular cell tumor of the vulva: six new cases. Arch Gynecol Obstet. 2006;273:246-9.
-
3Gross VL, Lynfield Y. Multiple cutaneous granular cell tumors: a case report and review of the literature. Cutis. 2002;69:343-6.
-
4Sehgal VN, Verma P, Sharma S. An asymptomatic, progressive, ulcerative nodule over the anterolateral surface of the left upper arm. Clin Exp Dermatol. 2012;37:695-7.
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5Brandão M, Domenech J, Noya M, Sampaio C, Almeida MVC, Guimarães MS, et al.Tumor de células granulares no pé (tumor de Abrikossoff): localização infreqüente de tumor relativamente raro (Foot granular cell tumor (Abrikossoff's tumor): unusual location of a relatively uncommon tumor). An Bras Dermatol. 2001;76:215-222.
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6Rekhi B, Jambhekar NA. Morphologic spectrum, immunohistochemical analysis, and clinical features of a series of granular cell tumors of soft tissues: a study from a tertiary referral cancer center. Ann Diagn Pathol. 2010;14:162-7.
-
7Torrijos-Aguilar A, Alegre-de Miquel V, Pitarch-Bort G, Mercader-García P, Fortea-Baixauli JM.. Cutaneous granular cell tumor: a clinical and pathologic analysis of 34 cases. Actas Dermosifiliogr. 2009;100:126-32.
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*
Work performed at the Dermatology Service at Pará Federal University (UFPA) (UFPA) - Belém (PA), Brazil.
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Financial funding: None
Publication Dates
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Publication in this collection
Nov-Dec 2013
History
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Received
12 May 2013 -
Accepted
13 June 2013