Malignant Schwannoma Arising in Patients with Von Recklinghausen's Disease: Report of Two Cases and the Comparison of Mast Cells between Benign and Malignant Portions

We described two cases of malignant schwannoma arising in patients with von Recklinghausen's disease and examined the mast cells infiltrated into histologic sections. One of the two cases histologically revealed apparent mast cell infiltration in some areas of malignant schwannoma as well as in the benign neurofibroma. The malignant lesion demonstrated significantly increased percentages of degranulated mast cells over the benign lesion using FITC-avidin staining. In an electron microscopic study, mast cells in the malignant lesion displayed empty granules, piecemeal degranulation, and canaliculi structures suggesting activation. These findings were not observed in the benign lesion. The other patient histologically showed no mast cells in the malignant lesion, although the benign neurofibroma in the patient disclosed numerous mast cells. The first patient had neither recurrence nor distant metastasis. On the other hand, the second patient without mast cells in the histology had multiple distant metastases. These findings raise questions about the role of mast cells in malignant schwannoma.     

A case of pencil core granuloma with an unusual temporal profile

The patient is a 47-year-old female with a bluish tumor resembling malignant melanoma at the macroscopic level on the medial aspect of her left big toe. The patient had crushed a pencil with this toe about 30 years previously and, since then, a bluish lesion had been present. About 15 years ago, the lesion had suddenly increased in size over the course of a few months. Subsequently, the size of the lesion had not changed noticeably. Ultrasonography, but not magnetic resonance imaging was a helpful preoperative examination to distinguish the lesion from malignant melanoma. The excised lesion contained a piece of material that resembled pencil lead and bluish mud. X-ray microanalysis of the lead-like material revealed that its composition was similar to that of pencil lead. Histologic examination showed features of foreign-body reaction, except for necrotic change and few histiocytes.     

Primary Malignant Melanoma of the Penis

A 50-year-old man had a primary malignant melanoma of the penis associated with granuloma telangiectaticum in the pigmented lesion. The patient had an operation for phimosis at the age of 20 and after the operation, he noticed a pigmented lesion at the operation site. We examined the literature and found 16 cases of primary malignant melanoma of the penis in Japan.     

Occult Basal Cell Carcinoma Arising in Seborrheic Keratosis

The present study reports a case of an occult basal cell carcinoma that arose in seborrheic keratosis. The patient was a fifty-six-year-old male who presented with a dark brown plaque on his back. Clinically, the lesion demonstrated no nodules or ulcerations suggesting that it was malignant. However, histopathological analysis of the lesion revealed an atypical basaloid cell mass that appeared to be a solid basal cell carcinoma beneath and surrounded by a seborrheic keratosis lesion. Thus, the coexistence of basal cell carcinoma and seborrheic keratosis is possible and should be considered when a malignant change in seborrheic keratosis is apparent.     

Malignant blue nevus

Blue nevus represents an aberrant collection of functioning benign dermal melanocytes. Its malignant degeneration is rare and is regarded as a form of malignant melanoma. We report a case of 35-year old male with this rare condition whose primary lesion over left foot ulcerated and patient later succumbed to multiple metastases.     

Multinodular keratoacanthoma and T cell lymphoma

This is a report of a case of multinodular keratoacanthoma (MNKA), a rare variety of solitary keratoacanthoma (KA) characterized by a collection of multiple KA nodules on the margins of a necrotic ulcerative lesion spreading centrifugally for 3 weeks in a patient with malignant non-Hodgkin's T-cell lymphoma. The very infiltrative nature of the main lesion made differential diagnosis difficult in the presence of extremely differentiated squamous cell carcinoma (SCC). Later, a KA lesion was to appear opposite a cutaneous lymphoma. The authors discuss the close links between the MNKA and the KA lesions and the immunosuppressed state of the patient.     

Primary cutaneous peripheral T-cell lymphoma,not otherwise specified,associated with lymphomatoid papulosis after a 9-year follow up: A case report

Lymphomatoid papulosis (LyP) is a self-limiting cutaneous T-cell lymphoproliferative disorder that may progress into malignant lymphoma. Most of the previously reported associated lymphomas are primary cutaneous anaplastic large-cell lymphoma and mycosis fungoides with a low mortality rate. We report a case of primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS), associated with LyP after long-term follow up. The patient was a 79-year old Japanese man followed up for 9 years. He suddenly developed a 3-cm ulcerated lesion on his forehead, which was diagnosed as an exacerbation of LyP. The lesion regressed after conservative treatment, but the patient soon developed multifocal pcPTCL-NOS. Thereafter, the patient developed pneumonia and cerebral infarction and died within a few months of the onset of malignant lymphoma. Aggressive cutaneous lymphoma may develop in LyP patients. The present case re-emphasizes the need for careful follow up of patients with persistent LyP.     

Disseminated malignant melanoma

A 25-year-old man had multiple asymptomatic, nodular lesions on the trunk, extremities and the face for 3 months. He also had left facial palsy with severe headache and vomiting. There were no other systemic or constitutional symptoms. Skin biopsy from a nodular lesion showed features of malignant melanoma, confirmed by Fontana Masson and S-100 protein staining. A diagnosis of disseminated malignant melanoma was made and the patient was treated symptomatically. The patient died in 4 months.     

Regressing atypical histiocytosis. Report of a case

A patient with a pseudomalignant, histiocytic dermal lesion is reported. Despite a torpid course, the lesion was considered on histologic grounds by several consultants to be malignant. It appears to be distinct from previously reported pseudosarcomas or pseudolymphomas and most closely resembles a recently described condition termed "regressing atypical histiocytosis." The clinical and histopathologic features are summarized.     

恶性透明细胞汗腺瘤

报告1例恶性透明细胞汗腺瘤。患者男,78岁。枕部黄豆大结节1年余,增大3个月,不痛不痒,在当地医院第一次活检未明确诊断,活检术后2个月复发。手术切除后组织病理诊断为恶性透明细胞汗腺瘤。半年后患者死亡,死亡原因不明。     

Malignant eccrine spiradenoma: an unusual presentation

An unusual case of a 60-year-woman with a progressively increasing painless, ulcerated plaque-like lesion in her right groin is described. The lesion was associated with multiple satellite nodules involving the right thigh with massive lymphoedema oft he involved limb. Histology established the diagnosis of an eccrine spiradenoma with malignant transformation in a satellite nodule. Wide surgical excision was not possible and the patient was subjected to palliative radiotherapy.     

Spontaneous partial regression of primary melanoma with death due to metastases

A 42-year-old man had a metastatic melanoma in the brain, but no obvious primary melanoma could be detected. A halo nevus-like depigmented lesion was found on the left side of the patient's back. The histopathologic features of the depigmented lesion were compatible with those of a spontaneously regressed primary lesion of malignant melanoma. Enhanced immune responses to autologous and homologous melanoma cells were not detected when investigated in the late disease stage. Subsequently, the patient died of disseminated melanoma.     

Solitary cutaneous metastasis on the buttock: a disclosing sign of pancreatic adenocarcinoma

A 48-year-old man, previously healthy and asymptomatic, showed a unique skin lesion located on the right buttock. The histopathological study of the lesion disclosed an adenocarcinoma metastatic to the skin. The primary tumour was found at the head of the pancreas, and was confirmed by biopsy. Six months after the beginning of treatment with Gemcitabine the patient is still asymptomatic, and the cutaneous lesion has disappeared. Carcinomas of the pancreas represent less than 5% of human malignant neoplasms, skin involvement is rare, and metastasis generally multiple and situated in the periumbilical area. To our knowledge, very few patients have been reported with a solitary cutaneous lesion disclosing a pancreatic adenocarcinoma. The atypical location, the absence of related symptoms or analytical disorders, and the good progress of the patient make this case especially interesting.     

Malignant transformation of multiple familial trichoepithelioma: case report and literature review

Patients with the autosomal-dominant form of multiple familial trichoepithelioma develop numerous tumours on the face, neck and upper trunk, beginning in childhood. Malignant transformation of such lesions is quite rare; only one case of "malignant trichoepithelioma" has been reported previously, inferring pilomatrix carcinoma on a histological observation. We report here the case of a patient who developed a malignant neoplasm in a long-standing trichoepithelioma lesion on her buttock. Histopathology revealed a transformation zone between the trichoepithelioma and a malignant tumour mass. This case also showed several features of a malignant neoplasm of trichoblastic origin.     

Nevus sebaceous and syringocystadenoma papilliferum.

A 52-year-old patient was admitted to the hosptial for evaluation of hypertension. He had two skin lesions, one on the forehead and one in the postauricular area, which had been present since birth. The forehead lesion was a nevus sebaceous and the postauricular lesion was a syringocystadenoma papilliferum. Except for a few patients with widespread nevus sebaceous and syringocystadenoma papilliferum associated with neurologic abnormalities, most of the previously reported patients with these nevi have had solitary lesions of one or the other. An association of nevus sebaceous and syringocystadenoma papilliferum in the same lesion is not uncommon. Despite bleeding and crusting in one of the lesions and despite informing the patient that a malignant neoplasm may develop in these nevi, he refused excision of either of the lesions.     

无黑素性黑素瘤伴全身转移1例

报道无黑素性黑素瘤全身转移1例。患者男 ,52岁 ,农民。左下肢有一米粒大小皮色肿物10年 ,增长缓慢。5个月前在基层医院行手术切除后很快发生局部淋巴结转移和全身转移。组织病理见真皮内瘤细胞呈团块状或巢状 ,小血管内可见肿瘤细胞浸润 ,HE染色未见黑色素。免疫病理S100( ) ,HMB45( )。     

Epidermodysplasia verruciformis and malignant thymoma

We describe a patient with a long history of skin lesions clinically and histologically consistent with epidermodysplasia verruciformis (EV) who developed malignant thymoma. HPV-9DNA was found in the macular lesion and HPV-4DNA and HPV-9DNA in the coexistent common warts. Thrombocytopenia and hypogamma-globulinaemia preceded the diagnosis of thymoma. Our patient seems to represent an example of an EV-like syndrome in immunodeficiency.     

Metastatic spinal cord compression from basal cell carcinoma of the skin treated with surgical decompression and vismodegib: case report and review of Hedgehog signalling pathway inhibition in advanced basal cell carcinoma

We report a case of a 66‐year‐old man with locally advanced and metastatic basal cell carcinoma (BCC) causing spinal cord compression, which was treated with spinal surgery and subsequent vismodegib. The patient presented with a large fungating chest wall lesion and a metastasis in T8 that was causing cord compression. He had neurosurgical decompression of the T8 lesion and fixation of the spine. Punch biopsy from the fungating chest wall lesion showed a BCC with some malignant squamous differentiation (basosquamous). Histopathological examination of the metastatic lesion in T8 at the time of surgical decompression identified features identical to the punch biopsy. The patient was referred to the oncology clinic for adjuvant treatment. In light of his metastatic disease and the large area over his chest wall that could not fully be covered by radiotherapy, he was treated with the novel oral Hedgehog signalling pathway (HHSP) inhibitor vismodegib, which led to marked improvement.     

Multiple myeloma with scleroderma-like changes

We present a case of a patient who presented concomitantly with generalized pruritus, brownish sclerodermatous plaques, sclerodactyly and a monoclonal band for IgG-kappa. The patient was diagnosed as having multiple myeloma by bone marrow examination. The rapidly progressive evolution with acute anuric renal failure, malignant hypertension and the skin sclerosis seem to be related to the neoplastic disorder. The scleroderma-like changes have to be differentiated from systemic scleroderma.