Type B lymphomatoid papulosis in a 12-year-old child.

In the WHO-EORTC classification (6) lymphomatoid papulosis (LP) is included among the primary cutaneous lymphomas; this term refers to non-Hodgkin’s lymphomas which at the time of diagnosis do not present extracutaneous manifestations; after the gastro-enteric system, the skin is the most frequent site of extranodal lymphomas (5). Primary cutaneous lymphomas (PCL), despite being made up of a cell population comparable to that of lymph node lymphomas, have a clinical behavior significantly different from lymph node lymphomas localized secondarily in the skin, because they remain confined to the skin for a long time or always and often regress spontaneously; therefore PCLs require a different therapy. PCLs are distinguished in T-cell PCLs, which make up 80%, and B-cell PCLs. Among the T-cell PCLs, after mycosis fungoides, CD30+ lymphoproliferative disorders (6) are most common: the latter include LP and anaplastic large cell lymphoma, that represent parts of the same disease spectrum, are not histologically distinguishable and differ essentially because skin lesions do not heal spontaneously in lymphoma unlike those in lymphomatoid papulosis. The primary lesion of LP is a papule that can enlarge to become a nodule; this usually does not exceed 2 cm in diameter; papules and nodules can become exuding and ulcerate; they usually come in subintrant crops of 1-10 elements and spontaneously regress in 3-6 weeks (1). LP can last from a few months up to 40 years (5). In contrast to the rather monomorphic and repetitive clinical features, histology shows highly variable patterns that recall different types of primitive cutaneous T lymphomas; in the same patient different histological types can be observed at the same time or at a later time (3). The medium-term prognosis of LP is good with a 10-year survival close to 100% (5). The risk of developing another lymphoma in adults with lymphomatoid papulosis is 10-20% (2) and mycosis fungoides is the most frequently associated lymphoma. In children, the risk is lower: in a recent meta-analysis of 251 children with lymphomatoid papulosis (4), the incidence of lymphomas is 5.6%, but the most frequent lymphoma is anaplastic large cell primary cutaneous lymphoma. In most cases, symptomatic therapy is sufficient; topical corticosteroids may be useful in lesions of exposed areas to limit the duration of cosmetic damage

Atypical vascular proliferation in chronic radiodermatitis secondary to radiotherapy for infantile hemangioma.

Radiotherapy is still used today in the treatment of infantile hemangioma (IH) in the case of particular localizations such as the pituitary fossa, when other treatments are not possible (1). On the other hand, it is not actually indicated in cutaneous IH, even if it was still used until the ’60s. Among its side effects there is chronic radiodermatitis (6), which today is observed mainly in women undergoing radiotherapy for breast cancer. The possibility of developing radiodermatitis depends on various factors, including the proximity to the skin of the radiotherapy target, the radiation energy, the overall dose of radiation and its treatment scheme, and the size of the skin surface exposed to radiation. Chronic radiodermatitis is histologically characterized by fibrosis of the dermis with disappearance of the hair follicles and glands, and rarefaction of the vessels; some residual vessels can be very dilated. The epidermis can be acanthotic or atrophic and characterized by numerous dyskeratotic cells. However, a more fearful side effect of radiotherapy for IH is the late onset, even after 6 decades (8) of tumors (2, 3, 7), including angiosarcoma (5) and melanoma (4). Besides malignant tumors there are also benign proliferations. The atypical vascular proliferations on radiodermatitis are benign tumors consisting of branched and dilated vessels or irregular vascular lacunae delimited by endothelium; degeneration into angiosarcoma has been described in 3-6% of cases (8). The considerable delay between irradiation and the appearance of these proliferations seems to be linked to the low doses of X-rays used in the treatment of hemangioma (8). The current case was presented due to the rarity of atypical vascular proliferations and to remind us that neoformations on radiodermatitis are not always malignant

Congenital circumscribed basaloid follicular hamartoma.

Basaloid follicular hamartoma is characterized by well-defined pathological findings, consisting of strands of basaloid cells connected with the hair follicles and immersed in a concentric loose stroma. It differs from basal cell carcinoma for the absence of clefts between the neoformation and the stroma and for the lack of mitosis and nuclear atypia (2). On the other hand, the clinical features are extremely variable going from generalized forms with autosomal dominant transmission, often associated with skeletal malformations, to nevoid forms distributed along the lines of Blaschko, to congenital, as in our case, or acquired circumscribed lesions. The individual lesions have no clinical characteristics (1), as they may be papules, nodules, plaques or dyschromic lesions, so the diagnosis often comes from the histological examination

Follicular mucinosis in an 11-year-old boy.

Follicular mucinosis (FM) is a histological term which means accumulation of mucin, i.e. mucopolysaccharides or glucosaminoglycans, in the outer sheath of the hair follicle and in the sebaceous gland; this histological picture can be found, in addition to idiopathic FM, in numerous benign conditions, such as lupus erythematosus (8), eosinophilic folliculitis (4), epidermal nevi (7), leprosy (3), and malignant disorders as follicular lymphomatoid papulosis (6) and especially folliculotropic mycosis fungoides (FMYF). The equivalent term of alopecia mucinosa, which introduces a clinical sign – alopecia – seems more appropriate to define the disease. However, alopecia is not always evident, especially when is not affected an area of skin with terminal hair. Follicular mucinosis presents clinically with clustered skin-colored papules or erythematous, sometimes scaling plaques, in which follicular reliefs may be evident. Based on the clinical course, we distinguish a form with one or a few lesions that resolve spontaneously within a few months, a form with more numerous elements, of more variable morphology that recur for many years and a form associated with lymphoma, generally with mycosis fungoides. The most important clinical problem is the distinction between idiopathic FM and FM associated with lymphoma, especially mycosis fungoides (1, 8). FMYF is the most frequent lymphoma associated with FM. However, the frequency of the association varies according to the different Authors between 9.4 and 64% of all cases of FM (1). Although FMYF can occur several years after the diagnosis of FM, in most cases FM and FMYF occur simultaneously (1). With regard to the association MF-FMYF children and more generally young patients and female subjects are less likely to have an associated FMYF (5). Most Authors agree that the onset of FM in the head with one or a few lesions has a benign clinical course (2). Monoclonality and TCR gene rearrangement, although more frequent in FMYF-associated forms of FM, do not represent a sure sign of malignancy (1). The present case was presented for its rarity; the presence of a single head lesion in a young boy and the polyclonal nature of the infiltrate speak in favor of a benign course, not excluding the need for careful clinical monitorin

Lobular capillary hemangioma in a 3 month old baby: early or congenital?

The differential diagnosis between infantile hemangioma and pyogenic granuloma is usually easy, especially due to the different time of onset. However, when PG arises in the first months of life or is even congenital (2), the diagnosis, which is important from a prognostic and therapeutic point of view, can present some difficulties. The immunohistochemical criterion of the positivity of GLUT-1 is considered an important discriminating criterion because PG is negative unlike IH, which is positive. However, this criterion, which is very useful for scientific studies, is less useful in clinical practice, because to use it you have to do a biopsy or removal, practices that are not usually performed in IH; and when removal is performed, it means that the physician has already clinically decided that it is PG and in this case the negativity of GLUT-1 only serves to confirm the clinical diagnosis of PG. Therefore the decision on the therapeutic behavior to be adopted in front of a vascular tumor remains essentially clinical. In some borderline cases, such as the current one, the most important clinical criteria are the implant basis, ulceration and bleeding. PG is usually pedunculated while IH is sessile, at least initially: only rarely, when IH is very large and is located in areas of loose skin, it can become pedunculated with time due to gravity. The most important clinical criterion (1) remains ulceration with consequent bleeding: IH rarely ulcerates, especially in the diaper region, and when ulcerating, bleeding is almost never massive. On the other hand, ulceration, being linked to its rapid growth, is the rule in PG unless it is localized in areas of thick epidermis such as in the palmar region; and the bleeding resulting from ulceration is usually copious, alarming the parents and prompting the doctor to surgically intervene

DERMATOFIBROSARCOMA PROTUBERANS IN A 5-YEAR-OLD CHILD.

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing but progressive soft tissue fibroblastic sarcoma with a high tendency to relapse, but with very low metastatic potential. In children it is even rarer than in adults with an estimated incidence of 1 per million per year (5). How-ever, there are also congenital cases (3). In the child there is no different distribution between the two sexes and it is localized in the limbs in two thirds of cases, in the trunk in a third (4). The histological examination is decisive for the diagnosis, which is based on the presence of a proliferation of fibroblastic appearance with a storiform arrangement, poor peripheral delimitation, invasion of the subcutaneous tissue, positivity of proliferating cells with CD-34, low mitotic index. The histological examination also serves to differentiate DFSP from fibrosaroma, which has a worse prognosis due to its ability to metastasize. Once the histological examination has confirmed the clinical suspicion of DFSP, the therapeutic problem arises. After evaluating the extent of the neoplasm with MRI or CT scan, it is still necessary to remove the tumor, if a biopsy has been done, or to reoperate on the scar if the tumor has been already removed, with a margin of 2 cm (1). Relapses are less frequent in the child. However, they occur between 9 (2) and 15% (4) after complete remission, attested by imaging and histologically free margins. For this reason, even after a complete remission, it is necessary to monitor the child for years. In advanced, unresectable or metastasizing cases, therapy with imatinib has been tried (2). The latter is a monoclonal antibody directed against the platelet-derived growth factor (PDGF), which is characteristically dysregulated in DFSP, due to a chromosomal translocation t(17;22), whereby the PDGF-beta gene on chromosome 22 fuses with the alpha collagen gene of type 1 (COL1A1) on chromosome 17. The translocation is also used in diagnosis because it is almost constantly present in DFSP (4). The prognosis of DFSP is good especially in children with 100% survival at 5 years (4). The current case has been described for its rarity and to reiterate the need for timely radical excision with sufficiently large margins and prolonged follow-up in affected children

"Black Ovaries": An Uncommon Case of First Systemic Recurrence of Melanoma

Here we describe a rare case of a 48-year-old woman with a previous history of malignant melanoma (pT2a pathological stage, IB clinical stage) occurring about five years previously. She complained of abdominal pain and pelvic discomfort, diagnosed as a consequence of a bilateral ovarian solid masses completely occupying the recto-uterine space. She underwent laparotomy surgery with total hysterectomy and bilateral salpingo-oophorectomy. Gross examination of the excised material revealed an unusual grey-black bilateral ovarian tumor; a histopathological diagnosis of ovarian bilateral metastatic melanoma was made. Imaging study (CT/MRI) did not reveal metastasis in other zones of the body. Melanoma metastasis usually affects the skin, liver, brain and lungs, and rarely gynecological localizations. On the other hand, most bilateral ovarian tumors are comprised of serous carcinoma, mature teratoma and gastrointestinal carcinoma metastasis. Exceptionally, primary ovarian melanoma may arise in mature ovarian cystic teratomas

Parental fitness questioned on the grounds of narcolepsy: Presentation of two cases

We report two cases of fathers whose parental fitness was questioned during divorce and custody litigation because of narcolepsy type 2 and type 1, respectively. These cases highlighted both the existence of a narcolepsy-related stigma and the need to involve sleep experts in custody assessments when concerns about the parental fitness are related to a sleep disorder, expanding the field of interest of the growing âsleep forensics"

Recurrent cutaneous leiomyosarcoma of the inner thigh

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Peritoneal Tuberculosis Mimicking Ovarian Cancer: Gynecologic Ultrasound Evaluation with Histopathological Confirmation

Peritoneal tuberculosis (TBP) is a very rare condition, accounting for about 1–2% of all tuberculosis cases. The diagnosis of TBP can be easily mistaken for advanced ovarian cancer (AOC) or peritoneal carcinoma because of overlapping laboratory and clinical findings. We reported the ultrasound characteristics of a case of TBP in a 67-year-old woman who presented to our institute with a 1-month history of intermittent lower abdominal pain, fever, and asthenia. Overall, 20 biopsy-retrieved specimen histopathological features were suggestive of peritoneal tuberculosis. Gynecologic ultrasound revealed increased adnexa with multiple nodular formations spread across the surface, suggestive of caseous nodules. Although this is a rare occurrence, clinicians should consider TBP as a differential diagnosis of ovarian or peritoneal cancer