Seguimiento de pilomatrixoma maligno metastásico agresivo mediante PET/TC con 18F-FDG / Aggressive metastatic malignant pilomatrixoma followed by 18F-FDG PET/CT

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IMAGING DIAGNOSIS-NUCLEAR SCINTIGRAPHIC, RADIOLOGICAL, AND PATHOLOGIC CHARACTERISTICS OF METASTATIC PILOMATRICOMA IN A DOG.

A 6-year-old castrated Goldendoodle dog was presented for left-sided lameness of 3 weeks' duration. Focal, moderate to marked increased 99m Tc-methylene diphosphonate (99m Tc-MDP) uptake was detected in the right caudal lung lobe, caudal angle of the left scapula, and the distal aspect of the left femur with whole body bone phase scintigraphy. Radiographs identified a well-circumscribed, oval-shaped soft tissue opaque mass in the right caudal lung lobe; a suspect oval-shaped osteolytic lesion in the proximal third of the left scapula; and an osteolytic lesion in the distal aspect of the left femur. Metastatic pilomatricoma was confirmed histologically at all three sites.

Malignant pilomatricoma in a dog.

An 11-year-old female German shepherd dog was presented with a history of lameness and pain in the left forelimb. Clinical examination revealed ataxia of the hindlimbs and a subcutaneous mass in the left prescapular region. Radiography revealed metastatic foci in the left humerus, lung and abdomen. Gross necropsy examination revealed a firm, white mass in the left prescapular region. Multiple nodules with similar characteristics were observed in the lung, liver and spleen. Bone lysis was noted in the humerus and the fifth to seventh lumbar vertebrae. Microscopical examination revealed a proliferation of basal cells forming irregular islands of various sizes and surrounding extensive zones of keratinized 'ghost' cells. A definitive diagnosis of malignant pilomatricoma was made. This is a rare tumour in dogs with no previous report of metastasis to the spleen and liver.

[Malignant pilomatrixoma of head and neck. A case report]. / Le pilomatricome malin cervical métastatique : à propos d'un cas.

We report the case of a 66-year-old patient with a pilomatrix carcinoma from the right submandibular region with pulmonary and cerebral metastases. The histological confirmation of the diagnosis has been based on the biopsy of the submandibular tumefaction which was rapidly growing. We tried at first a doxorubicine and cisplatine chemotherapy because of the considerable locoregional extension and the existence of pulmonary metastases. The patient response to three cures of chemotherapy was spectacular with a partial clinical response (75%) and a partially cleaned-up chest observed in the radiological evaluation. In the 5th cycle of chemotherapy following the same protocol, the patient presented a relapse with cerebral metastases. The patient received hypofractionated radiotherapy on the brain followed by etoposide and cisplatine chemotherapy, then oral vinorelbine. The patient died of progressive disease after 32 weeks.

Pilomatrix carcinoma with visceral metastases: case report and review of the literature.

Pilomatrix carcinoma, the malignant equivalent of pilomatrixoma, is rare among skin cancers. In the literature, there have been 80 cases of pilomatrix carcinoma reported, and among them nine were with metastases. The clinical presentation of this case is suggestive for the biology and of the usual history of this neoplasm. The patient was a 53-year-old male who had been treated 2 years earlier for a pilomatrix carcinoma located in the posterior part of the neck. The clinical presentation had been characterised by sudden paraplegia caused by vertebral collapse at T4 due to bone metastases. The patient underwent a first surgery for vertebral stabilisation and medullary decompression; then, he had a second operation for the resection of the local relapse of the tumour. Literature review and analysis of this case show that the pilomatrix carcinoma should be regarded as a highly locally aggressive tumour, with a high rate of local recurrence as well as metastases.

Malignant pilar tumor of the scalp: a case report and review of literature.

Pilar tumor is a rare neoplasm arising from the external root sheath of the hair follicle and is most commonly observed on the scalp. These tumors are largely benign, often cystic, and are characterized by trichilemmal keratinization. Wide local excision has been the standard treatment. Recent reports have described a rare malignant variant with an aggressive clinical course and a propensity for nodal and distant metastases which, therefore, merits aggressive treatment. In this report, we present a case of malignant pilar tumor of the scalp with multiple nodal metastases at presentation. Diagnostic and therapeutic considerations, in the form of adjuvant radiotherapy, are subsequently discussed.

Pilomatrix carcinoma with lymph node and pulmonary metastases.

CASE REPORT: A 72-year-old male patient developed a locally recurrent pilomatrix carcinoma (PC) of the right upper shank. Within a 2-year period he also developed bilateral pulmonary as well as inguinal, cervical and paraaortic lymph node metastases. After external-beam radiation therapy (EBRT) alone, sole interstitial high-dose-rate (HDR) brachytherapy (BRT) or postoperative EBRT with interstitial HDR-BRT boost, the patient demonstrated regressive or stable disease at the lymphatic sites. Systemic chemotherapy with intravenous paclitaxel failed to assure a substantial pulmonary response. In the course of the disease after 28 months pulmonary progression has led to continuing clinical deterioration. CONCLUSION: In accordance with literature data the hitherto course of this case corroborates that radiation therapy (RT) modalities can play an important role in the treatment of PC.

Pilomatrix carcinoma with lymph node metastases.

Pilomatrix carcinoma is a rare skin tumor with an origin from hair matrix cells. The tumor is locally aggressive with a great tendency for recurrence, but the metastatic potential is limited. A pilomatrix carcinoma in 76-year-old female with lymph node metastases is presented. In addition to classical histopathological criteria and DNA ploidy analysis, a broad panel of antibodies was used for evaluation of the metastatic potential. Both primary tumor and lymph node metastasis revealed extremely high proliferation and apoptotic rates. High constant expressions of CD44v6 and P-cadherin were also observed. In the metastasis, significant reduction of E-cadherin and beta-catenin was detected. The best approach for assessment of metastatic potential of pilomatrix carcinoma seems to be the complex evaluation of routine histological criteria like vessel invasion, mitotic index, apoptotic count, and new molecular markers of cell death and adhesion.

Pilomatrix carcinoma with lymph node and pulmonary metastasis: report of a case arising on the knee.

A 58-year-old woman presented with a pilomatrix carcinoma on the right knee. The tumor developed at the site of a previous lesion that had been present since she was 30 years old. Histologic study showed the presence of basaloid cells with numerous atypical mitoses, shadow cells, and calcification. After several surgical excisions, the tumor mass infiltrated the subcutaneous tissue, muscle, and bone, resulting in inguinal lymph node and pulmonary metastasis. We also review the literature and comment on the histopathologic differences from other cutaneous tumors.

Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature.

Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.

Pilomatrix carcinoma with multiple visceral metastases. Report of a case.

BACKGROUND: The malignant variant of pilomatrixoma is pilomatrix carcinoma, a low-grade, malignant lesion with a tendency to recur. Only three cases with visceral metastases, occurring some years after primary diagnosis, have been reported. METHODS: A case of metastatic pilomatrix carcinoma of the neck in a patient, age 50 years, is presented. RESULTS: Histologic examination of the cutaneous lesion showed a dense infiltrate of basaloid cells, an abrupt transition to shadow cells, and central necrosis. Two months after primary diagnosis, pulmonary and brain metastases developed. Despite chemotherapy and irradiation, the patient died a widespread disease 18 months after the primary diagnosis. An autopsy confirmed the diagnosis of pilomatrix carcinoma with metastases to several organs including the heart, lung, brain, liver, pancreas, kidney, adrenal gland, gastric and colorectal submucosa, skin, and bone. CONCLUSIONS: Pilomatrix carcinoma is very rare and usually behaves like a low-grade, malignant lesion with a tendency to recur. This patient's tumor is unique because of its highly aggressive behavior and visceral metastases.

Malignant pilomatrixoma of the scalp.

A 33 yr old man with a history of pilomatrixoma of the scalp, presented 5 mths later with a metastatic undifferentiated carcinoma in a submandibular lymph node. The cutaneous and lymph node tumors showed close histological similarity and features of malignancy. Investigations over 30 mths excluded a primary neoplasm elsewhere and consequently led to the diagnosis of malignant pilomatrixoma. These tumors are rare and most often located on the scalp. Most of the affected patients are middle age males. Wide excision is advised to avoid recurrences.

Clinical and histopathologic spectrum of pilomatricomas in adults.

BACKGROUND: Pilomatricomas are benign cutaneous neoplasms with differentiation toward hair matrix. Although previously reported to occur mostly in children and young adults, Taaffe et al. recently observed a second onset peak in adults and the elderly. METHODS: To study the clinical and histopathologic features of pilomatricomas in adults we analyzed files and histologic sections of all cases of pilomatricomas seen at the Department of Dermatology, University of Graz, Graz, Austria, between January 1980 and December 1990. RESULTS: A total of 118 patients were identified, 58 of whom were > 45 years of age. The clinical features of patients > 45 years of age were generally similar to those of patients < 45 years of age. Differential diagnoses in both groups were also comparable except in some cases of the older study-group where differential diagnoses included basal cell carcinoma, keratoacanthoma, and metastasis. Recurrence of the lesions after simple excision was observed in one patient > 45 years of age. Histopathologically, lesions in both age groups showed similar features except in four lesions of the older patients where "atypical" features were present. The histopathologic differential diagnosis of the lesions with "atypical" features included pilomatrical carcinoma, basal cell carcinoma with matrical differentiation, and matricoma. CONCLUSIONS: Our study reveals the relatively frequent occurrence of pilomatricomas in adults and the elderly. Pilomatricoma should be considered along with other benign and malignant conditions in the clinical differential diagnosis of solitary, firm skin nodules presenting in adults and the elderly, especially on the head and neck.

Pilomatrical carcinosarcoma of the cheek with subsequent pulmonary metastases. A case report.

We report a case of malignant pilomatrixoma with a pronounced biphenotypic morphology. The lesion, which was excised from the cheek of a 36-year-old man, was composed of a large pilomatrixoma lying within a spindled, sarcomatoid stroma. Fourteen months later, the tumor metastasized to the right upper lobe of the lung. We describe the tumor's pathology, histology, and immunochemistry and discuss the differential diagnosis. We also speculate on its histogenesis.