Acantholytic squamous cell carcinoma arising in actinic keratoacanthoma: Two cases with E-cadherin expression supporting a potentially different prognosis / Carcinoma de células escamosas acantolítico originado en queratoacantoma actínico: dos casos con expresión de E-cadherina indicando un pronóstico diferente

Among crateriform squamous proliferation, keratoacanthoma is considered as a squamous cell carcinoma or as a non-malignant, self-healing lesion that frequently becomes malignant. To date, published cases of neoplasms originating from keratoacanthoma are always conventional squamous cell carcinomas. Acantholytic squamous cell carcinoma arising in keratoacanthomas has not been previously reported. We present two crater-shaped nodular lesions on the face of elderly patients with clinical and histopathological features of keratoacanthoma with transformation areas to acantholytic squamous cell carcinoma. In the immunohistochemical study, Ki-67 and p63 immunostaining supports the diagnosis of acantholytic squamous cell carcinoma ex-keratoacanthoma. We suggest that E-cadherin expression and vimentin negativity could probably be related with less aggressive behaviour and a better prognosis (AU)

Dentro de las proliferaciones escamosas crateriformes, el queratoacantoma se considera como un carcinoma escamoso o como una lesión benigna autocurativa que frecuentemente se vuelve maligna. Las neoplasias descritas que se originan del queratoacantoma son siempre carcinomas escamosos convencionales. Carcinomas escamosos acantolíticos originados en queratoacantomas no se han descrito en la literatura. Presentamos 2 nódulos crateriformes en la cara de ancianos con características clínicas e histopatológicas de queratoacantoma con áreas de transformación a carcinoma escamoso acantolítico. En el estudio inmunohistoquímico, la expresión de Ki-67 y p63 apoya el diagnóstico de carcinoma escamoso acantolítico ex-queratoacantoma. Nosotros hipotetizamos que la expresión de E-cadherina y la negatividad de vimentina están probablemente relacionadas con menor agresividad y excelente pronóstico en estos pacientes (AU)

Esprúe colágeno: ¿entidad secundaria o independiente de la enfermedad celíaca? / Collagenous sprue: secondary or independent from celiac disease?

El esprúe colágeno es una entidad poco prevalente que cursa con diarrea persistente con pérdida de peso y malabsorción por afectación del intestino delgado, principalmente duodeno y yeyuno proximal, necesitando para el diagnóstico la presencia de una clínica y una histología compatible con atrofia y depósito subepitelial de colágeno. Su etiología no es totalmente conocida, aunque su origen más propuesto es el autoinmune, ya que está ampliamente relacionada con la enfermedad celíaca e incluso se ha propuesto que se trate de una evolución de celiaquía refractaria a dieta sin gluten. En relación a esta incertidumbre presentamos el caso de una paciente con diarrea malabsortiva e importante repercusión clínica por esprúe colágeno, la cual tuvo una buena respuesta a corticoides orales (prednisona), pero hubo que añadir azatioprina. Además mejoró inicialmente con nutrición parenteral central domiciliaria...

Collagenous sprue is a rare disease that goes with persistent diarrhea, weight loss and bad absortion, because it affects the small intestine, mainly duodenum and proximal jejunum. Diagnosis is made by having clinical signs and histological proof of atrophy and subepitelial deposit of collagenous material. Its etiology is not known completely, it is proposed that the origin is autoimmune because its relationship with celiac disease. Also there is a proposal that is a celiac evolution to gluten free diet. Is because this is not clear that we present a case of a patient with bad absorptive diarrhea and a clinical expression of collagenous sprue, that had a great clinical response to corticosteroids (prednisone) but we had to add azatioprine. Also, initially improved with home parenteral nutrition center...

[Collagenous sprue: secondary or independent from celiac disease?]. / Esprúe colágeno: ¿entidad secundaria o independiente de la enfermedad celíaca?

Collagenous sprue is a rare disease that goes with persistent diarrhea, weight loss and bad absortion, because it affects the small intestine, mainly duodenum and proximal jejunum. Diagnosis is made by having clinical signs and histological proof of atrophy and subepitelial deposit of collagenous material. Its etiology is not known completely, it is proposed that the origin is autoimmune because its relationship with celiac disease. Also there is a proposal that is a celiac evolution to gluten free diet. Is because this is not clear that we present a case of a patient with bad absorptive diarrhea and a clinical expression of collagenous sprue, that had a great clinical response to corticosteroids with home parenteral nutrition center.

Postradiation cutaneous pleomorphic rhabdomyosarcoma with extracellular collagen deposits reminiscent of so-called amianthoid fibers.

Rhabdomyosarcoma is a malignant mesenchymal neoplasm that rarely presents as primary skin tumor. So-called amianthoid fibers are hyalinized collagen mats that have been described in myofibroblastic tumors but not in rhabdomyosarcoma. A 65-year-old male developed a submandibular nodule 9 years after an oral squamous cell carcinoma, which had been treated with chemotherapy and radiotherapy. Histological examination of the nodule revealed a pleomorphic rhabdomyosarcoma with extracellular collagen deposits reminiscent of so-called amianthoid fibers. By immunohistochemistry, the tumor cells were positive for vimentin, desmin, smooth muscle actin (SMA), muscle-specific actin (MSA), CD10, CD56, CD99, ß-catenin and D2-40. However, only 15-20% of the tumor cells were positive for myoglobin, MyoD1 and myf-4/myogenin. We describe first so-called amianthoid fibers harboring blood capillaries in a rhabdomyosarcoma, suggesting that they are rigid collagen structures that lead to tumor vascularization. The low expression for myogenic regulatory proteins and strong expression for other markers may be misleading and do not contribute to the diagnosis of rhabdomyosarcoma.

Sclerosing dermatofibrosarcoma protuberans (DFSP): an unusual variant with focus on the histopathologic differential diagnosis.

A 59-year-old man presented with a 10-cm x 8-cm tumoral plaque with a superficial nodule in the interscapular region of the back (Fig. 1). The lesion had been growing for 25 years. As a cystic lesion was suspected, the superficial nodule was biopsied. The histopathologic diagnosis was low-grade sarcoma with sclerosis. Two months after the initial biopsy, the lesion was completely excised, reaching the muscular fascia, with a 2-cm margin and with a free graft. Formalin-fixed paraffin-embedded samples were submitted to histologic and immunohistochemical study (4-microm paraffin sections); frozen tissue was submitted to electron microscopy. For histopathology, sections were stained with hematoxylin and eosin. Immunohistochemistry was performed following standard avidin-biotin immunoperoxidase procedures with primary antibodies for vimentin, CD34, smooth muscle-specific actin, bcl-2, S-100, desmin, myoglobin, factor VIII, p53 (all from DAKO, Copenhagen, Denmark), HHF-35 (Enzo Diagnostics, Farmingdale NY), cytokeratin (AE1/AE3) (Biogenex, San Ramon, CA), and factor XIIIa (Calbiochem Novabiochem Corporation, La Jolla, CA). At low magnification, the histologic study of the initial tumoral nodule revealed a poorly circumscribed mesenchymal proliferation, with fibroblastic-like neoplastic cells arranged in a fascicular and storiform pattern, admixed with extensive areas of sclerosis. At higher magnification, tumoral cells were spindle-shaped with hyperchromatic nuclei and scant cytoplasm. In some areas, sclerosis was so evident that a keloid-like pattern was seen (Fig. 2a). The surgical specimen showed a fibroblastic neoplastic proliferation infiltrating the dermis and hypodermis. In the dermis, cells were arranged in a storiform pattern, whereas in the hypodermis there was a honeycomb or lace-like pattern (Fig. 2b). There were also cellular areas alternating with sclerotic areas, with transitional zones in between, in both the dermis and hypodermis. The immunohistochemical study of the initial tumoral nodule and the surgical specimen showed that tumoral cells expressed vimentin, CD34 (Fig. 3), bcl-2, HHF-35, and smooth muscle actin. Neoplastic cells failed to show positivity with desmin, myoglobin, factor XIIIa, factor VIII, S-100, cytokeratin (AE1/AE3), and p53. An ultrastructural study revealed spindle cells having an irregular contour with a well-developed granular reticulum endoplasmic (REG) system in their cytoplasm, as well as some Golgi complexes and mitochondria. Also visible was the presence of many actin filaments and some myosin condensations (Fig. 4), characteristics of a fibroblastic cell with myofibroblastic differentiation. The final histopathologic diagnosis of the surgical specimen was sclerosing dermatofibrosarcoma protuberans. Two years after surgery, the patient is alive and well.