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Soft Tissues
Published in Joseph Kovi, Hung Dinh Duong, Frozen Section In Surgical Pathology: An Atlas, 2019
A 50-year-old man was admitted to the hospital complaining of a tender lump in his left buttock of 5 months duration. On exploration a deeply situated, 8.0 by 5.0 by 5.0 cm sized, firm, pink-gray circumscribed mass was found next to the gluteus maximus muscle. An incisional biopsy was performed and was submitted for frozen section study. Microscopically, the neoplasm was highly cellular. In some areas predominantly spindle cells were seen. These occasionally formed a “storiform” pattern. Both normal and abnormal mitotic figures were common. In other regions of the tumor there were noted a large number of rather bizarre-looking giant cells. These were either mononucleated or multinucleated. Also scattered pigment-laden macrophages were apparent (Figures 43 and 44).
Pleomorphic Sarcoma
Published in Dongyou Liu, Tumors and Cancers, 2017
Pleomorphic sarcoma (UPS) is usually a large and deep-seated tumor of 20 cm (retroperitoneal location) with a fibrous or fleshy cut surface, necrosis, or myxoid change. Histologically, the classic form of UPS shows frequent transitions from storiform to pleomorphic areas. Storiform areas consist of plump spindle cells arranged in short fascicles in a cartwheel, or storiform, pattern (i.e., cells emanating from a central focus) around slit-like vessels. Pleomorphic areas contain plumper fibroblastic cells and more rounded histiocyte-like cells arranged haphazardly with no particular orientation to vessels. High cellularity, marked nuclear pleomorphism, abundant mitotic activity (including atypical mitoses), chronic inflammatory cells, and necrosis (in high-grade lesions) are observed. Immunohistochemically, pleomorphic sarcoma is positive for vimentin, α-1-antitrypsin, α-1-antichymotrypsin, factor XIIIa, CD68, CD10, CD34, and CD99 (35%), but negative for keratin, melanocytic markers, CD45, S100, and muscle markers [7].
Tumors of Smooth Muscle
Published in Omar P. Sangueza, Sara Moradi Tuchayi, Parisa Mansoori, Saleha A. Aldawsari, Amir Al-Dabagh, Amany A. Fathaddin, Steven R. Feldman, Dermatopathology Primer of Cutaneous Tumors, 2015
Dermatofibrosarcoma protuberans: Widely infiltrative and monotonous cellsStoriform pattern
Deep fibrous histiocytoma of the index finger: a case report
Published in Case Reports in Plastic Surgery and Hand Surgery, 2023
Hiroki Shibayama, Yuichiro Matsui, Daisuke Kawamura, Daisuke Momma, Takeshi Endo, Yuki Matsui, Yasutaka Yawaka, Kanako C. Hatanaka, Emi Takakuwa, Hirokazu Sugino, Yutaka Hatanaka, Tadashi Hasegawa, Norimasa Iwasaki
HE staining showed that the tumor was relatively well-circumscribed and contained no cutaneous adnexa (Figure 2(b, c)). The lesions had a high cellular density rather than fibrous tissues, with spindle-shaped cells mainly seen in a storiform pattern and partially in a short fascicular pattern. There was no nuclear pleomorphism or hyperchromasia, and no staghorn vessels. The lesions did not have giant cells, hemosiderin, a hemangiopericytoma-like vascular pattern, or a slit-like pattern. Immunohistochemical staining revealed CD68 and factor XIIIa-positive histiocytes (Figure 2(d, e)). CD34 was negative and α-smooth muscle actin (α-SMA) was positive (Figure 2(f)). However, the signal transducer and activator of transcription 6 (STAT6), S100 protein, and desmin were negative. On the basis of these findings, we ruled out fibroma of the tendon sheath, tenosynovial giant cell tumor, DFSP, and SFT, and the diagnosis of DFH was confirmed. Dermatofibroma was ruled out by findings of the normal overlying skin, easy dissection from the skin and no cutaneous adnexa in HE staining. Because DFH rarely occurs in the hand, we performed additional cytogenetic analysis.
Malignancy post-hematopoietic stem cell transplant in patients with primary immunodeficiency
Published in Expert Review of Clinical Immunology, 2020
Kesserwan et al. reported an association between Dermatofibrosarcoma protuberans (DFSP) and ADA deficient SCID patients. DFSP is normally a particularly rare malignant skin tumor in childhood with a low risk of metastasis [58]. The characteristic histologic finding is a spindle cell tumor with a storiform pattern which is CD34+ . At the cytogenetic level it is associated with a characteristic chromosomal translocation (t[17;22] [q22;q13]) resulting in the COL1A1-platelet-derived growth factor β (PDGFB) fusion gene. Kesserwan et al. described 8 patients with DFSP lesions. Mostly these presented as multiple small brown atrophic plaques, less than 1 cm in diameter which had been present in some cases since birth. Cases have been reported post-treatment with PEG-ADA and HSCT suggesting that despite immunological correction of the underlying disorder this association remains a risk [58–60]. Toxic metabolites in the skin and an increased propensity for DNA strand breaks in patients with ADA deficiency may be involved as mechanisms for this association. Careful histological and cytogenetic testing of lesions is required to make the diagnosis and treatment requires wide excision with margin control due to the infiltrative nature of the lesions.
Neonatal Gastrointestinal Stromal Tumor of the Sigmoid Colon: A Case Report and Review of Literature
Published in Fetal and Pediatric Pathology, 2020
Mostafa Kotb, Marwa Abdelaziz, Marwa Beyaly, Mohamed Mekawy, Hayssam Rashwan, Nagwa Mashali
Grossly, an intestinal segment measuring 5 cm in length showed on section a soft fleshy grayish white tissue measuring 3x2x2cm fungating from and infiltrating the wall of the intestine (Figure 1(A and B)). Microscopic examination revealed a colonic wall lined by mucosa showing ulceration of the lining epithelium and infiltration of the wall by a non-capsulated tumor formed of a spindle cellular population. The cells were arranged in interlacing fascicles with occasional storiform pattern. The cells were spindle-shaped or round vesicular with spindle shaped nuclei and scant cytoplasm. The mitotic count was <5/50HPFs. No areas of necrosis were noted. Clusters of colonic crypts were seen entrapped within the tumor. The tumor infiltrated the muscularis propria and extended to the serosal fat (Figure 2). Both surgical resection margins were free of tumor tissue.